Ear Related Conditions PDF

9/26/23 UNOFFICIAL UNOFFICIAL...

9/26/23 UNOFFICIAL UNOFFICIAL LEARNING AIMS Learn the different conditions, diseases, and afflictions of the outer, middle, & EAR RELATED inner ear CONDITIONS Understand the potential mechanism of certain diseases and discover how they Kevin M. Logan, BSSP, MClinAud are usually managed or treated 2023 Ear conditions and diseases 2 1 2 UNOFFICIAL UNOFFICIAL 1. Structural/anatomic abnormalities DEVELOPMENTAL-RELATED ABNORMALITIES OF THE OUTER EAR a. Microtia & anotia OUTER EAR b. Aural atresia c. Acquired/mechanical MICROTIA & ANOTIA 2. Ear blockages Microtia is a congenital deformity of one or both external ears (the pinna or auricle), usually causing a. Foreign bodies it to be smaller. Anotia is the absence or underdevelopment of the external ear. b. Earwax Reconstructive surgery (called pinnaplasty or otoplasty) can often restore the appearance of a near normal ear and can include prosthetics 3. Otitis externa a. Bacterial infection and otomycosis (fungal infection) The accompanying atresia in this case signals the presence of substantial conductive hearing loss, b. Malignant external otitis (aka necrotizing otitis externa) leading which may include an absent middle ear cavity. When the hearing loss cannot be corrected surgically, to osteomyelitis the loss may be addressed through a bone-conduction hearing aid or implant. c. Dermatitis – eczema, psoriasis d. Furunculosis (boils) and perichondritis 4. Ear tumours and growths a. Osteomas and exostoses as non-cancerous bone growths b. Cysts c. Keloids 2023 Outer ear conditions 3 2023 Outer ear conditions 4 3 4 1  9/26/23 UNOFFICIAL UNOFFICIAL DEVELOPMENTAL-RELATED ABNORMALITIES OF THE OUTER EAR ACQUIRED/TRAUMATIC INJURY OF THE OUTER EAR AURAL ATRESIA a congenital condition characterized by the absence or incomplete development of the Involves the loss or damage of the external ear external ear canal (the ear canal leading from the external ear to the middle ear). The ear due to various reasons. canal may be narrowed, blocked, or completely absent, which can result in various degrees These include motor vehicle accidents, sports of conductive hearing loss. injuries, assaults, burns, falls, animal bites and It can affect one or both ears and vary in severity (or grades) injury, workplace injuries, among others. Treatment for ear atresia may involve surgical interventions such as canaloplasty or bone- Affectations depend on the severity of the injury anchored hearing aids (BAHA). and site Treatment options are generally similar if hearing is affected 2023 Outer ear conditions 5 2023 Outer ear conditions 6 5 6 UNOFFICIAL UNOFFICIAL EXTERNAL EAR BLOCKAGES EXTERNAL EAR BLOCKAGES FOREIGN BODIES CERUMEN IMPACTION (EARWAX BLOCKAGE) Refers to any object or substance that has entered and Occurs when an excessive amount of earwax (cerumen) become lodged in the ear canal. builds up in the ear canal and becomes impacted or obstructed. Common in children who may insert small objects like beads, buttons, or small toys into their ears. Can cause a conductive hearing loss if there is a total occlusion and may also lead to otalgia, tinnitus, or vertigo. It can also accidentally enter the ear canal in adults, Can be caused by several factors, including the use of cotton such as through accidents, falls, other activities, or swabs or other objects that push earwax deeper into the ear even insects that may enter and get stuck in the ears canal, naturally occurring ear canal shapes that make it It may cause otalgia (ear pain), hearing loss, more prone to impaction, or overproduction of earwax. inflammation, discharge, tinnitus, and etc. 2023 Outer ear conditions 7 2023 Outer ear conditions 8 7 8 2  9/26/23 UNOFFICIAL UNOFFICIAL OTITIS EXTERNA OTITIS EXTERNA BACTERIAL OR FUNGAL INFECTION BACTERIAL OR FUNGAL INFECTION often referred to as "swimmer's ear.” because repeated It can also happen from allergic reactions or exposure to water can increase its risk. damaging the skin in the ear canal. a condition characterized by inflammation or infection Common symptoms of otitis externa include ear pain of the ear canal. It typically occurs when water or discomfort, itching, redness, and drainage from remains trapped in the ear canal after swimming or the ear (otorrhea). The ear canal may also appear bathing, creating a moist environment that is red & swollen, and there can be a temporary conducive to the growth of bacteria or fungi. decrease in hearing. 2023 Outer ear conditions 9 2023 Outer ear conditions 10 9 10 UNOFFICIAL UNOFFICIAL OTITIS EXTERNA OTITIS EXTERNA MALIGNANT OTITIS EXTERNA DERMATITIS Also known as necrotizing otitis externa Could be included under otitis externa as it is sometimes called eczematous otitis externa. A severe and potentially life-threatening infection that primarily affects the external ear canal and the surrounding Condition characterized by inflammation and irritation of the skin tissues. lining the external ear canal. It may also be the result of eczema or psoriasis. Characterized by the spread of infection beyond the ear canal into the adjacent structures, including the bones of the skull Common symptoms of aural dermatitis include itching, redness, base (osteomyelitis – bone inflammation). swelling, and flaking or scaling of the skin within the ear canal. In some cases, it may also lead to pain or discomfort. typically occurs in older adults with weakened immune systems or underlying health conditions like diabetes. The infection can Treatment typically involves identifying and addressing the lead to severe complications, including cranial nerve palsies, underlying cause, which may include avoiding irritants or allergens, meningitis, and bone destruction keeping the ear dry, and using topical corticosteroid creams or ear drops. 2023 Outer ear conditions 11 2023 Outer ear conditions 12 11 12 3  9/26/23 UNOFFICIAL UNOFFICIAL OTITIS EXTERNA EAR TUMOURS AND GROWTHS FURUNCULOSIS (BOILS) AND PERICHONDRITIS OSTEOMAS AND EXOSTOSES Furunculosis is a medical term used to describe the formation Osteomas are benign bony growths that can develop in the of one or multiple boils or abscesses in the skin. A boil, also ear canal itself or on the surface of the bony portion of the known as a furuncle, is a painful, pus-filled bump that forms ear canal. These growths are composed of mature bone when a hair follicle or oil gland becomes infected with tissue and are typically solitary, slow-growing, bacteria. pedunculated, and non-cancerous. Perichondritis is a condition characterized by inflammation and infection of the perichondrium, which is the thin layer of Exostosis is also a bony growth that develops on the surface connective tissue that surrounds and provides blood supply of a bone but differs with osteomas from its histopathologic to the cartilage in the body, including the cartilage of the features. Exostoses are also typically benign and often pinna. It can cause the ‘cauliflower ear’ appearance. covered by a layer of cartilage, exhibiting broad based Both are commonly caused by the Staphylococcus aureus elevations of bone involving the tympanic bone. They are bacteria. usually multiple, bilaterally symmetrical. 2023 Outer ear conditions 13 2023 Outer ear conditions 14 13 14 UNOFFICIAL UNOFFICIAL EAR TUMOURS AND GROWTHS EAR TUMOURS AND GROWTHS OSTEOMAS AND EXOSTOSES KELOIDS AND CYSTS Exostoses can be triggered by prolonged Ear keloids are abnormal growths of scar tissue that can form in response to an injury or trauma to the ear, including ear exposure to cold water as a protective response piercings, surgery, or other forms of ear trauma. Keloids are a to repeated exposure to cold water. type of hypertrophic scar, but they differ from typical scars in that they tend to grow beyond the boundaries of the original Treatment is typically considered when these wound and can become raised, thick, and firm growths cause significant symptoms or complications, such as repeated ear infections or Ear cysts are fluid-filled or semi-solid sacs that can develop canal blockage. Surgical removal may be necessary in or around the ear. They are typically non-cancerous and in some cases to alleviate symptoms or improve can vary in size from small to relatively large. There are hearing. several types of cysts that can affect the ear but the most common one would be sebaceous cysts. 2023 Outer ear conditions 15 2023 Outer ear conditions 16 15 16 4  9/26/23 UNOFFICIAL UNOFFICIAL MIDDLE EAR EARDRUM PERFORATIONS 1. Eardrum perforations Openings or tears in the tympanic membrane which Direct physical trauma 2. Tympanosclerosis separates the external ear canal from the middle ear. Barotrauma and Acoustic trauma 3. Negative middle ear pressure Hearing loss may occur as the eardrum is unable to a. Valsalva and Toynbee manoeuvre effectively transmit sound vibrations from the external ear to the middle ear and ossicular chain. Other symptoms 4. Otitis media could include ear pain, discharge (clear, bloody or pus- a. Kinds: acute, suppurative (with effusion), chronic suppurative filled), tinnitus, or vertigo b. Retraction pockets and cholesteatoma Can heal on their own but larger or persistent perforations c. Mastoiditis may require treatment from antibiotic ear drops to d. Facial palsy surgical repair (tympanoplasty/myringoplasty) to restore the integrity of the eardrum. e. Patulous eustachian tube Can occur due to various causes including 5. Otosclerosis Infections 2023 Middle ear conditions 17 2023 Middle ear conditions 18 17 18 UNOFFICIAL UNOFFICIAL TYMPANOSCLEROSIS NEGATIVE MIDDLE EAR PRESSURE a condition characterized by the presence of white, Common characteristics may include: A condition characterized by an imbalance in the a eustachian tube dysfunction) secondary to chalky calcium deposits or scar tissue that can pressure between the middle ear and the outside infections or allergies or blockage of the tube by Reduced Hearing environment. enlarged adenoids form on the tympanic membrane. These deposits can affect the normal functioning of the middle ear Calcium Deposits Normally, the middle ear should be at approximately and hearing. Scar Tissue the same pressure as the surrounding atmosphere. In Typically associated with chronic or recurrent ear No Pain or Infection cases of negative middle ear pressure, the pressure infections, particularly otitis media with effusion within the middle ear becomes lower than the (fluid buildup in the middle ear). The condition may atmospheric pressure because the tissue in the develop as a response to the inflammation and middle ear space absorbs the air inside the cavity. healing processes in the middle ear. This causes the eardrums to retract inwards into the Hearing aids may be recommended or surgical cavity and may interfere with the normal vibration interventions, such as a tympanoplasty (eardrum of the membrane to produce a conductive loss repair surgery), may be considered Usually caused by oedema of the eustachian tube (or 2023 Middle ear conditions 19 2023 Middle ear conditions 20 19 20 5  9/26/23 UNOFFICIAL UNOFFICIAL NEGATIVE MIDDLE EAR PRESSURE PATULOUS EUSTACHIAN TUBE Common symptoms of negative middle ear pressure Patulous Eustachian tube (PET) - a condition may include ear fullness or a plugged feeling, characterized by the Eustachian tube remaining open in reduced hearing or muffled sounds, ear pain or the resting state (when it is normally closed). PET is a discomfort, and tinnitus type of Eustachian tube dysfunction. In some cases, it may lead to recurrent ear infections. Treatment involves addressing the underlying cause. Use of decongestants or antihistamines for allergies Major symptoms include autophony (hearing one's own or respiratory infections, or practicing techniques to voice, breathing, or heartbeat), echoing which may help open and equalize pressure in the Eustachian interfere with speech production, wave-like sounds, and tube a sensation of fullness in the ear. Misophonia (strong dislike or hatred of certain sounds) and hypersensitivity The two usual techniques used are the Valsalva to sounds may occur. manoeuvre (depicted on the diagram) and the Toynbee manoeuvre which is the pinch and swallow Pregnancy, weight loss, and some neurologic disorders (air) (such as stroke) are thought to be amongst the most common predisposing factors 2023 Middle ear conditions 21 2023 Middle ear conditions 22 21 22 UNOFFICIAL UNOFFICIAL OTITIS MEDIA OTITIS MEDIA An infection can come from bacteria (such as Streptococcus Essentially means an inflammation of the middle ear – it is characterised by recurrent or persistent ear discharge pneumoniae & Haemophilus influenzae) or virus (such as the a spectrum of diseases and one of the most common (otorrhoea) over 2 to 6 weeks through a perforation of the condition called herpes zoster oticus) diseases in children. It can resolve spontaneously but may tympanic membrane. CSOM usually begins as a lead to permanent hearing impairment and life-long complication of recurring AOM with perforation in Retraction pockets are a localized area of the TM consequences. childhood. invaginated (collapses) into the ME cavity. It is common in children and may result to erosion of the ossicular chain and Acute otitis media (AOM) results from infection by viruses contribute to cholesteatoma formation. or bacteria, often as a complication of the common cold or of allergies. Acute suggests that the condition is severe and Cholesteatoma – a benign collection of keratinized skin the onset is sudden. (squamous epithelium) cells trapped within the middle ear and forms a cyst that can grow. It is dangerous due to the Otitis media with effusion (OME) is a condition in which destructive enzymes and acid produced by the skin cells. The there is fluid in the middle ear but no signs of acute exact cause is unknown. infection. As fluid builds up in the middle ear and Eustachian tube, it places pressure on the tympanic membrane. Cholesteatomas can be congenital or acquired. They can CSOM erode into the bone and CNS and cause severe complications. Chronic suppurative otitis media (CSOM) is persistent It is difficult to cure once it becomes infected as the lesion inflammation of the middle ear or mastoid cavity. It is does not have blood supply and antibiotics cannot penetrate the mass. 2023 Middle ear conditions 23 2023 Middle ear conditions 24 23 24 6  9/26/23 UNOFFICIAL UNOFFICIAL OTITIS MEDIA OTITIS MEDIA For most of these infections, antibiotics and antivirals are indicated, depending on the results of the biopsy and extent of the infection. Corticosteroids are used to reduce Mastoiditis is the inflammation of a portion of inflammation and swelling. the temporal bone referred to as the mastoid air cells. It is a common complication of AOM. Certain medical procedures are also necessary such as Consequences can include meningitis and myringoplasty, mastoidectomy, nerve reconstruction, intracranial abscess if left untreated. In severe prosthesis and artificial barriers, among others. Grommets cases, mastoidectomy (removal of part of the are tiny ventilation tubes that are put inside the eardrum to mastoid bone) is recommended. prevent a build-up of negative pressure (in cases of ET dysfunction) or fluid (for acute or recurrent OME). Facial nerve palsy is a rare complication of acute suppurative otitis media when the With the advice of the otolaryngologist or specialist, bone conduction hearing aids (and sometimes air conduction infection reaches the facial nerve. Intravenous hearing devices) may be considered to address the long- antibiotics & corticosteroids, and physiotherapy term hearing loss that may occur due to the infection or may be required to treat it. medical procedures. 2023 Middle ear conditions 25 2023 Middle ear conditions 26 25 26 UNOFFICIAL UNOFFICIAL OTOSCLEROSIS OTOSCLEROSIS Symptoms include unilateral gradual decline in hearing a progressive ear condition that affects the small bones on one side followed by the other ear. Tinnitus and (ossicles) in the middle ear, usually the stapes bone. It dizziness are also common. typically affects both ears, although one ear may be more Can show up on an audiogram with the “Carhart’s notch” affected than the other. – a depression at 2kHz in bone conduction Abnormal bone growth occurs around the ossicles, and the Treatment options include: ossicular chain becomes ‘remodelled.’ Commonly, the stapes bone, becomes fixed or immobilized. Wait and see Hearing aids In the early stages of the disease, areas of soft bone appear around the inner ear in a process called Medications – fluoride is thought to slow down the “otospongiosis.” It can result in bone loss from the otic progression capsule and with time, the bone loss stops, and the soft Surgery called stapedectomy to replace the areas scar. This hard, scarred bone is called “otosclerosis” immobilized stapes bone with a prosthesis or stapedotomy, a less invasive surgical procedure, to The exact cause is not understood but it seems to have create a small hole in the stapes bone to improve genetic and hormonal factors. It is more common in women its mobility. and may worsen during pregnancy. 2023 Middle ear conditions 27 2023 Middle ear conditions 28 27 28 7  9/26/23 UNOFFICIAL UNOFFICIAL OSSICULAR CHAIN DISCONTINUITY SUMMARY TABLE Ossicular chain discontinuity or dislocation is the separation of the ME bones that results into a hearing loss. Aetiology Degree of Loss Audiometric Tympanogram type It can be caused by bone fractures (following a traumatic incident) and temporal bone or head trauma, eardrum perforation configuration and trauma, complications from severe otitis media and cholesteatoma Microtia/Anotia varies flat A Atresia Moderate-severe flat Not testable The most common type is thought to be the incudostapedial joint dislocation majority following a head trauma. Foreign bodies & total EAC Mild-moderate Flat/saucer B low volume occlusion Otitis externa Varies-mild Rising A/not testable Conservative approaches are usually taken and hearing aids may be recommended instead. If the conductive loss is lasting Exostoses Varies-mild to moderate Rising/flat A/B low volume more than 6 months, surgical intervention is advised. This may include ossiculoplasty which usually results in varying degrees TM perforation Mild Rising/flat B high volume of hearing loss recovery (air-bone gap closure) Negative middle ear Mild Rising/flat C pressure Ossicular chain discontinuity Moderate Flat Ad (deep) OME Mild-moderate Rising/flat B low volume CSOM Mild-moderately severe Rising/flat B/not testable Otosclerosis Mild-moderately severe Flat/Carhart’s notch As (shallow) Ossiculoplasty is the surgical restoration of the Hearing loss from outer and middle ear conditions are usually conductive in nature unless the extent of the condition spreads into the sound transmitting mechanism of the middle cochlea ear. A total or partial ossicular replacement prosthesis can be used (TORP/PORP) 2023 Middle ear conditions 29 2023 Middle ear conditions 30 29 30 UNOFFICIAL UNOFFICIAL INNER EAR (and beyond) COCHLEAR OTOSCLEROSIS 1. Cochlear otosclerosis a. BPPV b. Vestibular paroxysmia Cochlear otosclerosis is defined as otosclerosis located in the otic 2. Enlarged vestibular aqueducts capsule involving the cochlear endosteum and causing sensorineural 3. ‘Inflicted’ sensory loss 8. Cochlear nerve (CN) hypoplasia & aplasia hearing loss or mixed type hearing loss. a. Trauma and perilymphatic fistula 9. Presbycusis b. Noise-induced hearing loss 10. Hyperacusis c. Cochlear loss following surgical complications 11. Central Auditory Processing Disorders d. Ototoxic related hearing loss e. Radiation-induced hearing loss Bonus 4. Vestibular schwannoma Congenital & inherited hearing loss 5. Meniere’s disease a. Syndromes (Usher, FASD) 6. Infections and inflammations in the inner ear b. Infections (rubella, herpes simplex) Usually occurs in long standing cases of stapes fixation where the a. Labyrinthitis disease process invades the otic capsule and sometimes even the b. Vestibular Neuritis-Viral Neuronitis/Herpes semicircular canals. Zoster (shingles) c. Idiopathic sudden sensorineural hearing loss Sodium fluoride treatment can be tried to arrest the disease progress but d. Auto-immune inner-ear disease amplification of hearing with hearing aids is always considered 7. Other vestibular related diseases 2023 Middle ear conditions 31 2023 Inner ear conditions 32 31 32 8  9/26/23 UNOFFICIAL UNOFFICIAL PERILYMPHATIC FISTULA ENLARGED VESTIBULAR AQUEDUCT A perilymphatic fistula (PLF) is an abnormal communication between Vestibular aqueducts are narrow, bony canals that travel from the inner ear to deep the perilymph-filled inner ear and outside the inner ear that can allow inside the skull. Running through each vestibular aqueduct is a fluid-filled tube called perilymph to leak from the cochlea or vestibule, most commonly the endolymphatic duct, which connects the inner ear to a balloon-shaped structure through the round or oval window. called the endolymphatic sac. The functions of the endolymphatic duct and sac are not completely understood. High frequency hearing loss occurrs as an early symptom after barotrauma. Positional dizziness can occur a few hours after auditory Research suggests that most children with enlarged vestibular aqueducts (EVA) will develop some amount of hearing loss. However, scientists do not think that EVA causes symptoms. Nystagmus (repetitive uncontrolled eye movements) can be the hearing loss, but that both are caused by the same underlying defect. A vestibular present. Treatments include bed rest, tissue grafts via surgery to repair aqueduct is abnormally enlarged if it is larger than one millimeter. damaged membranes, and more recently, blood patch injections (as an experimental treatment) EVA may have many causes but the most well-known is mutations in a gene called SLC26A4 (previously known as the PDS gene). Two mutations in the SLC26A4 gene can Ear barotrauma is an injury caused by a difference in pressure between result in Pendred syndrome. the external ear canal and the middle ear. No treatment has proven effective in reducing the hearing loss associated with EVA or in slowing its progression. Surgical procedures are not recommended and may cause further harm. Avoidance of contact sports or activities that might lead to head injury is recommended. Hearing aids are indicated if it progresses into a hearing loss. 2023 Inner ear conditions 33 2023 Inner ear conditions 34 33 34 UNOFFICIAL UNOFFICIAL NOISE-INDUCED HEARING LOSS HEARING LOSS FROM INTRAOPERATIVE when sounds become excessively loud, even for a brief duration, or when they are persistently loud over an extended period, they have the potential to harm the delicate AND POSTOPERATIVE COMPLICATIONS structures within the inner ear. This can result in the development of noise-induced hearing loss (NIHL). It is completely preventable using sufficient hearing protection. Permanent hearing loss can result from a host of surgical procedures related to the ear during or after the actual operation. Depending on the procedure being done, it may Depending on the noise source and loudness, NIHL can be gradual and difficult to detect have different outcomes, presentations, and complications. at first or it can be sudden (e.g. gunshots or explosions). It may cause tinnitus or first be noticed as a temporary reduction in hearing. These are often associated with cochlear implantation or post-surgical wound infections. NIHL can also occur for procedures that involve bone/tumour removal near the ear After exposure to a significantly loud sound, the loss in hearing can be detected in pure- (from the grinding tools and related equipment). Related to this, even orthopaedic surgeons tone audiometry as a “temporary threshold shift” from one’s baseline hearing levels can have hearing loss- due to the power tools they used and the NIHL they receive from it! (Jeppesen and Faber, 2013) until it becomes a “permanent threshold shift” where the NIHL officially becomes lifelong. Hearing loss following non-ear related surgery could also happen, although rare. Postoperative sensorineural hearing loss (SNHL) following non-otologic surgery is often detected only by audiometric evaluation. This phenomenon has been reported following spinal anesthesia or lumbar puncture and can be unilateral or bilateral, typically affecting the low frequency range. The loss of cerebral spinal fluid and hence the drop in intracranial pressure is thought to Surgical complications following cochlear implantation in result in hearing loss and cranial nerve palsy. (Vilhena et al., 2016) adults based on a proposed reporting consensus by Jeppesen and Faber, 2013 2023 Inner ear conditions 35 2023 Inner ear conditions 36 35 36 9  9/26/23 UNOFFICIAL UNOFFICIAL OTOTOXICITY RADIATION-INDUCED LOSS It is the pharmacological adverse reaction affecting the inner ear or auditory Radiation therapy plays an integral part in the treatment of head and neck nerve, characterized by cochlear or vestibular dysfunction. It involves the cellular cancers. Given their central location, the ear and temporal bone are often in degeneration of cochlear and/or vestibular tissues leading to its functional radiation fields for nasopharyngeal, oropharyngeal, parotid, and periauricular deterioration, due to the usage of certain therapeutic agents skin cancers. Acute and late toxicities due to radiation are seen in all parts of the ear and hearing loss may occur when the temporal bone is included in radiation Some of the most common ototoxic medications are: aminoglycoside antibiotics, fields. It can present as conductive loss in cases of middle ear or eustachian tube platinum-based chemotherapeutic agents, loop diuretics, macrolide antibiotics, (ET) injuries or as SNHL when the inner ear is involved. (Nader and Gidley, 2019) and antimalarials. Radiation induced SNHL is a dose-dependent, progressive, permanent, and late The damage is usually permanent and decisions are made over what takes effect. Patients undergoing head and neck RT can develop SNHL, most often as a precedence for the person in question based on different health guidelines and result of cochlear injury (such as cell death and necrosis). Postradiation OME regulations. may also occur (labelled Fig. 2 on the left) Ototoxicity: A Challenge in Diagnosis and Treatment by Ganesan, Schmiedge et al., 2018 Images from Challenges of Hearing Rehabilitation after Radiation and Chemotherapy by Nader and Gidley, 2019 Tobramycin and gentamycin ototoxicity audiogram examples 2023 Inner ear conditions 37 2023 Inner ear conditions 38 37 38 UNOFFICIAL UNOFFICIAL ACOUSTIC NEUROMA MÉNIÈRE’S DISEASE also known as vestibular schwannoma A disorder of the inner ear that causes severe dizziness (vertigo), ringing in the ears (tinnitus), hearing loss, and a feeling of fullness or congestion in the ear. The symptoms and severity of a benign, usually slow-growing tumor that develops from the Ménière's disease vary widely from person to person and the exact cause is unknown. vestibulocochlear nerve. The tumour develops from the overproduction of Schwann cells—the cells that normally wrap around nerve fibers to In Ménière’s disease, the endolymph buildup in the labyrinth interferes with the normal balance and help support and insulate nerves. hearing signals between the inner ear and the brain. This abnormality causes vertigo and other symptoms of Ménière’s disease. It can affect the hearing and balance, usually causing unilateral (one- sided) or asymmetric hearing loss, tinnitus (ringing in the ear), and Ménière’s disease does not have a cure but there are some strategies dizziness/loss of balance. As the tumour grows, it can interfere with the used to manage its effects: faceial nerve (the trigeminal nerve), causing facial numbness, Prescription medications to reduce dizziness/vertigo. Prescription weakness, or paralysis on the same side. If the tumour becomes large, drugs such as meclizine, diazepam, glycopyrrolate, and it will eventually press against nearby brain structures (such as the lorazepam can help relieve dizziness and shorten the attack. brainstem and the cerebellum), becoming life-threatening. Diet restriction and diuretics. Limiting dietary salt and taking There are three options for managing a vestibular schwannoma: (1) diuretics (water pills) help some people control dizziness by surgical removal, (2) radiation, and (3) observation. If there is hearing reducing the amount of fluid the body retains, which may help lower fluid volume and pressure in the inner ear. Reducing loss, hearing aids are indicated. caffeine, alcohol, chocolate, and smoking is thought to help Inner ear conditions Other treatments like cognitive therapy, injections, or surgery 2023 39 2023 40 Inner ear conditions 39 40 10  9/26/23 UNOFFICIAL UNOFFICIAL LABYRINTHITIS VESTIBULAR NEURITIS Labyrinthitis is an inflammation of the membranous labyrinth of the inner While labyrinthitis refers to swelling of both branches of the ear and typically presents with vertigo, nausea, vomiting, tinnitus, and/or vestibulocochlear nerve (the vestibular and cochlear portions) or inner ear hearing loss. It is normally caused by a bacterial or viral infection; (labyrinth), vestibular neuritis or vestibular neuronitis refers to swelling of however, it can be a manifestation of systemic autoimmune disease or one branch of the vestibulocochlear nerve (the vestibular portion), which human immunodeficiency virus in some cases. affects balance. The condition is also thought to be viral in origin, in contrast with labyrinthitis that could also be bacterial. Viral: The most common cause is secondary to a viral upper respiratory tract infection. Maternal rubella or cytomegalovirus (CMV) infection is one of the most common When this nerve becomes inflamed or swollen, the signal is interrupted causes of congenital deafness with labyrinthitis. Ramsay-Hunt syndrome, also known as herpes zoster oticus, is caused when a latent varicella-zoster virus infection causing the brain read the information incorrectly. This results in dizziness, becomes reactivated. HIV is linked as well although not fully understood why or how. vertigo and other balance-related symptoms. Bacterial: typically arises from either bacterial meningitis or otitis media when it enters via the oval or round window. Syphilis is thought to be linked to labyrinthitis. It is considered a benign, self-limited condition that typically lasts several In the early stages of the disease, the clinical signs of vestibular neuritis are a horizontal torsional nystagmus. The eyes are days, but can take weeks to months for all vestibular symptoms to In rare cases, it can result in labyrinthitis ossificans, wherein pathological unable to stably fixate on an object during a ‘head impulse test’ completely resolve. The symptoms are typically constant, which differs which results into a phenomenon called ‘catch-up saccades’ new bone formation occurs within the membranous labyrinth – the cochlea where there is a delay/correction of the eyes when the head is from other peripheral conditions that are episodic like Meniere’s disease or ossifies. laterally rotated. BPPV. Treatment must be tailored to the etiology and symptoms. Anti-nausea Treatment usually consists of addressing the acute symptoms of dizziness medications can be prescribed as well as steroids to combat the via medications (antiemetics and antihistamines) followed by vestibular inflammation. rehabilitation therapies. 2023 Inner ear conditions 41 2023 42 Inner ear conditions 41 42 UNOFFICIAL UNOFFICIAL IDIOPATHIC SUDDEN SNHL AUTOIMMUNE INNER EAR DISEASE Idiopathic: arises suddenly and with unknown causes Defined as a condition of bilateral sensorineural hearing loss (SNHL), caused by an It is an unexplained, rapid loss of hearing either all at once or over a few days. SSNHL happens because there ‘uncontrolled’ immune system response where the body's immune system attacks cells in the is something wrong with the sensory organs of the inner ear. Sudden deafness frequently affects only one ear. inner ear that are mistaken for a virus or bacteria. It can happen in isolation (as just labyrinthine disease) or as part of other systemic autoimmune disorders. Steroids is thought to help with SSNHL as it reduces inflammation, decreases swelling, and helps the body fight illness. This is either administered orally in pill form or more preferably (due to reduced side effect risks) Will manifest as rapidly progressing, sensorineural hearing loss that may begin on one ear through an intratympanic injection into the middle ear. and quickly affects the other ear as well. Patients show a 30 dB, or higher, bilateral and asymmetrical SNHL, developing between 3-90 days (Ciorba et al, 2018). Vestibular symptoms Early treatment within 48 hours of onset of symptoms is ideal, while beyond 2 weeks it is considered no longer can have a fluctuating course during the active phase of disease. Additionally, tinnitus, as a effective (Henderson, Bennett, & Clark, 2020) ringing, roaring, or hissing noise, may be present. Diagnosis is difficult as it can mimic other ear disorders; it is essential to rule out other causes of progressive sensorineural hearing loss, such as acoustic neuroma, neurosyphilis, and Meniere’s, etc. The main treatment for this condition is corticosteroids but literature suggests that the estimated real efficacy of steroid treatment is only 14% among sufferers. When medical treatment is insufficient, hearing aids are advised (Ciorba et al, 2018). 2023 Inner ear conditions 43 2023 44 Inner ear conditions 43 44 11  9/26/23 UNOFFICIAL UNOFFICIAL VESTIBULAR PAROXYSMIA COCHLEAR NERVE DEFICIENCY Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia (absence). It an episodic vestibular disorder which usually presents with a high frequency of attacks which occurs due to compression of the is a rare malformation characterized by a hypoplastic (underdeveloped) or absent cochlear nerve. It can be accompanied with other associated eighth cranial nerve (vestibulocochlear nerve) by an artery. The inner ear malformations or syndromes. The degrees of hearing loss attacks are characterised as recurrent and spontaneous, spinning or non-spinning vertigo that generally last less than one minute resulting from CNH can range from moderate to profound depending on the quantity of nerve fibers present. and occur in a series of up to 30 or more per day. Vestibular paroxysmia can often be treated with low doses of a