When taking a history from a patient with suspected Labyrinthitis it is important to screen for risk factors. These include recent viral infections (commonly upper respiratory tract infections), cholesteatoma, meningitis, and acute/chronic otitis media.

• Nausea, vomiting and severe ‘room spinning’ vertigo are the most common symptoms.[1] Initial vertigo rarely lasts longer than 72 hours, however, balance issues and occasional brief episodes of vertigo may persist for a number of weeks.
• Patients may also complain of hearing loss or tinnitus, this clinically differentiates labyrinthitis from vestibular neuritis, which is not associated with any auditory symptoms.[19]
• Targetted questioning regarding neurological symptoms such as numbness, weakness, dysphagia, dysarthria, and facial pain is crucial as these could indicate a CVA affecting the brain stem.[4]
• It is also important to clarify the number of episodes of ‘room-spinning’ vertigo. If it has occurred more than once then Meniere disease must also be amongst the differentials.[25]

On examination, patients will often display nystagmus, the fast phase will move away from the affected ear. Patients may also present with gait and balance disturbances, therefore, both Romberg’s test and tandem gait are key to a complete examination. Rinne and Weber hearing tests will likely demonstrate sensorineural hearing loss in the affected ear. Otoscopy may give clues to the etiology of the disease e.g otitis media, cholesteatoma. A full neurological examination should be performed including cerebellar and meningeal tests. Finally, if querying bacterial meningitis be sure to fully examine the patient for the classical non-blanching rash.[26]

Audiometry is useful to confirm examination findings and assess the extent of the sensorineural hearing loss. Specific investigations into the vestibular system (e.g., evoked myogenic potentials, electronystagmography, and rotary chair tests) are not indicated in the acute phase of the disease. However, they can be useful in assessing long term compensation and residual deficits.

The laboratory tests should be tailored to the patients’ symptoms and differential diagnosis. If the patient presents with severe vomiting, then a urea and electrolytes panel should be performed to assess whether electrolyte replacement needs to be initiated. If bacterial meningitis is suspected, then CSF cultures should be sent. Subsequently, consider HIV and Syphilis serology in high-risk individuals or atypical presentations. Finally, consider autoimmune screens in patients with systemic symptoms or atypical presentations with negative serology.

MRI/ CT imaging may be useful. For example, 13% of acoustic neuromas present with sudden hearing loss, which can be diagnosed using an MRI. Gadolinium-enhanced magnetic resonance imaging (GdMRI) is extremely accurate in predicting if a patient with bacterial meningitis will subsequently develop hearing loss, a complication in 14% of cases.[27][28][29]

Treatment must be tailored to the etiology and symptoms. Viral labyrinthitis should principally be managed by hydration and bed rest in the outpatient setting. However, it is crucial that patients are given safety netting advice to seek further medical help if their symptoms deteriorate, or they experience any neurological disturbances (e.g., weakness/numbness, diplopia, slurred speech, and gait disturbance).[30] There is currently little evidence around the use of antiviral medications and steroids.[31][32]

In bacterial labyrinthitis, antibiotic type and route depend on the source. For example, if it is secondary to otitis media, then topical antibiotic drops should be prescribed. The ear must be cleaned if the patient has purulent otorrhea or tympanic membrane perforation. However, if the patient has systemic symptoms, consider oral or even intravenous antibiotics, depending on the severity of the infection. If bacterial meningitis is queried, then treat immediately with intravenous antibiotics. A concurrent otogenic source should still be ruled out, and topical, and IV antibiotics can be given simultaneously if appropriate.

The initial management of autoimmune labyrinthitis is corticosteroids. If patients are refractory to corticosteroid therapy, then other immunomodulators may be considered, such as azathioprine, etanercept, or cyclophosphamide. These agents are often used in chronic conditions due to their reduced side effect profile when compared to corticosteroids. However, this treatment should be overseen by a specialist.[33] If a patient’s serology is positive for syphilis or HIV, they should be started on the appropriate treatment and referred to a specialist.[34]

Whilst suffering from initial vertigo, patients will want to lie motionless with their eyes closed. it is important that they attempt to mobilize as soon as possible (even if this exacerbates their vertigo) as this is believed to help with vestibular compensation and prognosis.[35]

Benzodiazepines and antihistamines can be used to treat initial vertigo. However, symptoms should not persist for longer than 72 hours. Therefore, only short courses of these medications should be prescribed as they can inhibit vestibular compensation. Antiemetics, such as prochlorperazine, should be used to help control nausea and vomiting. Whilst patients with sudden hearing loss should receive a course of corticosteroids and be referred to a specialist.

A small minority of patients may be left with tinnitus. It is important to recognize the relationship between this and reactive depression and intervene early with treatments such as tinnitus re-training, tinnitus maskers, hearing aids, and/or biofeedback.[36][37]

Surgical intervention is only required in a minority of cases, for example, mastoidectomy in patients with cholesteatoma or severe mastoiditis. Occasionally patients may require drainage of effusions or myringotomy in labyrinthitis secondary to otitis media.

Once the acute labyrinthitis has resolved, patients may be left with persistent vestibular symptoms, which can seriously impact their life.[38] These patients should be referred for vestibular rehabilitation.[39]

•  Vestibular Neuritis: This has a similar presentation to Labyrinthitis without the hearing loss.[40]
•  Meniere disease: This also causes hearing loss and vertigo; however, the episodes are usually intermittent.[41]
•  Benign positional vertigo: This causes dizziness but not hearing loss, and patients will have a positive Dix-Hallpike test.[42]
•  Posterior fossa CVA: If a patient presents with any neurological signs, ataxia, hoarseness dysarthria, or dysphagia, a CT/MRI head should be requested immediately to rule out a CVA.[43]
•  Acoustic neuromas/ vestibular schwannomas: These can be visualized using a GdMRI.[44]
•  Inner ear malformations, e.g. (missing crura of the stapes, atresia, abnormal incus): This typically presents with progressive hearing loss and can be diagnosed using CT or MRI imaging.[45]
•  Temporal bone fracture: This should be considered if there is a recent history of head trauma and can be confirmed with CT imaging.[46]
•  Inner ear hemorrhage: Commonly associated with trauma and easily demonstrated on MRI.[47]
•  Temporal bone neoplasm: Usually presents with cranial nerve deficits or facial paralysis and should be investigated with MRI and/or CT.[48]
•  Multiple sclerosis: This is more likely to present alongside other systemic symptoms such as spasticity or signs of optic neuritis.[49]

Superoxide dismutase has been shown to limit the hearing loss and prevent Labyrinthitis Ossificans in gerbils with bacterial meningitis, and TNF-alpha inhibitors appear to reduce postmeningitic cochlear injury and hearing loss.[50][51] There is also some evidence suggesting that corticosteroid therapy in pneumococcal meningitis may prevent labyrinthitis ossificans and research into intra-tympanic corticosteroids as a superior therapeutic administration route have also shown promising results.[52] Finally, cochlear microperfusion and antioxidant therapy have shown some potential as adjuvant therapies.[53][54][55]

The acute vertigo of labyrinthitis should resolve within a couple of days; however, milder symptoms may persist for a number of weeks. Provided the patient had no serious neurological sequelae, the prognosis is usually good. However, patients with neurological complications may require further interventions. For example, ventriculoperitoneal shunts in patients with hydrocephalus secondary to bacterial meningitis.[56] Patients who receive a prolonged course of benzodiazepines and/or antihistamines to treat their vertigo appear to have delayed vestibular recovery. In contrast, suppurative labyrinthitis is more likely to cause permanent hearing impairment.

Bilateral vestibular hypofunction is a debilitating complication associated with bilateral labyrinthitis, most commonly caused by bacterial meningitis. It can result in visual impairment (Oscillopsia) and impaired spatial awareness often leaving patients reliant on mobility aids.[57] After severe cases of labyrinthitis, some patients may be left with hearing loss or tinnitus which can be managed with a hearing aid or tinnitus specific therapies.[36] 

Complete deafness is a rare complication of bilateral labyrinthitis, usually caused by bacterial meningitis. Labyrinthitis ossificans is recognized as a complication of suppurative labyrinthitis.[58] If bacterial labyrinthitis is not treated effectively there is the risk of it developing into mastoiditis. This typically responds well to IV antibiotics, however, it can result in mastoidectomy with tympanoplasty in severe cases.[59] Finally, labyrinthectomy may be required to terminate the disease process in rare cases of Labyrinthitis secondary to cholesteatoma.[59]

Labyrinthitis is often secondary to another infection, such as otitis media or meningitis. Therefore early diagnosis and effective management of these should prevent the development of labyrinthitis or at least minimize the risk of long term complications. It is also key that populations are up to date with their vaccinations to reduce the risk of contracting measles, mumps, or rubella.

The interdisciplinary team is crucial in delivering optimum care for a patient with labyrinthitis. Firstly, it is key that primary and secondary care doctors are well aware of both the signs and symptoms of labyrinthitis and the other common/ serious differentials diagnoses. This will ensure that the patient receives prompt and effective management, and potentially life-threatening conditions that can mimic labyrinthitis, such as cerebellar CVA, are not missed. 

Both nurses and pharmacists are key in the initial management of a patient with labyrinthitis and, therefore, must have a good understanding of the condition. For example, nursing staff must be aware that the patient could be at high risk of falls and may need assistance with transfers due to their vertigo. They may also take a lot longer completing tasks and, therefore, should not be rushed. Pharmacists should provide advice on the most appropriate medications to help treat the patient’s symptoms, e.g., nausea, and vertigo. For example, the use of benzodiazepines and antihistamines to manage acute vertigo should only be prescribed short term as they can hinder vestibular recovery. They should also be aware of medications that can cause symptoms that mimic those of labyrinthitis when taking a drug history, as this is a cause that is occasionally overlooked.

Patients can be left with residual vestibular symptoms, which severely impact their quality of life. This is when input from both occupational and physiotherapists is essential. Occupational therapists can perform assessments of the home environment and implement changes to improve patient safety and maintain quality of life. Whilst physiotherapists can offer vestibular rehabilitation. After 1 or 2 sessions of vestibular rehabilitation, symptoms can be significantly improved or may be eliminated altogether.