Labyrinthectomy
Joseph B. Nadol, Jr.
Michael J. McKenna
Unilateral surgical vestibular ablation is an effective treatment for unilateral peripheral vestibular dysfunction in the presence of ipsilateral severe profound sensorineural hearing loss in patients in whom medical management has failed to result in satisfactory control or compensation. Certainly the most common indication for this surgery includes unilateral Meniere’s disease. However, the procedure may also be indicated in unilateral peripheral vestibular dysfunction in other disorders, such as posttraumatic or postinfectious labyrinthitis, in which there is no useful cochlear function. The physiologic rationale for peripheral vestibular ablation is based on the fact that compensation for peripheral vestibular dysfunction seems to be more rapid when (i) unilateral vestibular dysfunction is fixed rather than fluctuating and (ii) the absence of unilateral peripheral input to vestibular stimuli is more easily compensated, compared with disordered vestibular input (1).
Unilateral vestibular ablation can be accomplished in a variety of ways, including section of the vestibular or cochleovestibular nerve, transmastoid labyrinthectomy, and transcanal labyrinthectomy. Unilateral eighth nerve section or selective vestibular nerve section was introduced by Dandy (2) in 1928 for Meniere’s disease, and transmastoid labyrinthectomy was described by Jansen (3) in 1895 for peripheral vestibulopathy caused by suppurative labyrinthitis. The transmastoid approach was applied to other peripheral vestibulopathies, including Meniere’s disease by Milligan (4) and Lake (5) in 1904. Pulec (6) emphasized the importance of total ablation of the peripheral vestibular apparatus, a tenet that has been adopted in modern surgical procedures for unilateral vestibular ablation.
A transcanal approach for a labyrinthectomy was introduced by Lempert (7) using an endaural exposure of the middle ear. A transcanal approach was popularized by Schuknecht (8) and Cawthorne (9). A modification of transmeatal labyrinthectomy with and without cochleovestibular neurectomy was described by Silverstein (10) to be more certain of complete peripheral vestibular ablation. The importance of total surgical ablation was also emphasized by Armstrong (11) and Ariagno (12).
PATIENT SELECTION FOR PERIPHERAL VESTIBULAR ABLATION
The patient’s vestibular episodes must be debilitating and ascribable to vestibular dysfunction in one ear, as indicated by preoperative evaluation. Reasonable attempts at medical management, including pharmacologic and rehabilitative measures, must have failed. Hearing loss in the affected ear should be in the severe to profound range, that is, with a pure-tone average of 75 dB or worse and speech discrimination of 20% or less. Particularly in Meniere’s disease, in which bilateral involvement may occur in 10% to 40% of patients (13,14), surgical intervention, especially in the form of labyrinthectomy, which will destroy all residual hearing, should be reserved as a last resort. Documentation that the vestibulopathy is debilitating is not always straightforward and depends on a variety of clinical and patient variables that include not only frequency, duration, and severity of vertiginous episodes but also such issues as age and occupation.
Preoperative Evaluation
A thorough history of the vestibular complaint, including severity, duration, frequency of attacks, and contributing factors, should be documented. The nonotologic medical history should clearly document other disorders that may compound the vestibulopathy or interfere with compensation once a labyrinthectomy is performed. These include age, other debilitating disorders, peripheral neuropathies as may be seen in diabetes, cardiac disease, proprioceptive disorders, generalized arthritis, visual disturbances such as cataract, and evaluation of any potential secondary gain. A complete otolaryngologic and head and neck examination should be performed, including a neurotologic examination with evaluation of cranial nerves, cerebellar testing, and office vestibular testing such as Dix-Hallpike maneuver. Behavioral audiometry, including pure-tone thresholds for air and bone conduction and speech discrimination, is essential. A fistula and Hennebert’s sign should be sought (15).
Formal vestibular testing should include, as a minimum, bilateral caloric function, preferably documented by electronystagmography. A complete vestibular evaluation using the rotating chair and posturography may give further clues and confirmation that the vestibulopathy may be ascribed to the suspect ear and that there is no evidence of significant bilateral vestibular dysfunction. Imaging may not be necessary in every case. However, given the fact that lesions in the posterior fossa may at times mimic the symptoms of Meniere’s syndrome, magnetic resonance imaging (MRI) with gadolinium is useful to rule out the presence of a cerebellopontine angle or other posterior fossa tumor or the presence of demyelinating lesions. Other neurologic dysfunction elicited by history or physical examination should be evaluated by referral to a neurologist. In some patients with Meniere’s disease, the caloric function in the affected ear will remain normal or minimally reduced. In such cases, labyrinthectomy may still be justified based on clearly localizing symptoms and signs such as fluctuating severe to profound sensorineural loss, ipsilateral tinnitus, coincidence of unilateral aural symptoms of fullness and tinnitus, and hearing loss concurrent with the vestibular attack. Preoperative vestibular testing is also useful for the purpose of counseling the patient. In general, the more active the caloric response in the affected ear, the more severe the postoperative reaction to labyrinthectomy may be.
In summary, the ideal candidate for a labyrinthectomy is an otherwise healthy patient with clear-cut unilateral vestibular dysfunction and ipsilateral severe to profound sensorineural hearing loss who has failed conventional medical management and has no secondary gain in remaining disabled (16). The entity of delayed endolymphatic hydrops in a previous deafened ear and the presence of Tumarkin drop attacks are generally excellent indications for labyrinthectomy if other patient selection criteria have been satisfied.
Preoperative Counseling of a Patient and Informed Consent
Preoperatively, the patient should be aware of the natural history of Meniere’s disease, including spontaneous remission rate of approximately 70% over 8 years (17) and the fact that Meniere’s disease may become bilateral in up to 40% of patients (13,14). The patient should be informed that all hearing in the operated ear will be lost, although the potential for cochlear implantation of the labyrinthectomized ear, if bilateral sensorineural loss occurred in the future, may be preserved. The patient should also be aware that tinnitus, which is almost universally present in unilateral Meniere’s disease, is not likely to be ameliorated by this procedure. It should be explained that postoperatively the patient, particularly one with relatively intact vestibular function in the affected ear, will suffer a severe and protracted episode of vertigo with nausea and vomiting lasting several days. A prolonged period of disequilibrium, possibly requiring vestibular physical therapy, may ensue, particularly in patients with contributing factors such as age, obesity, arthritis, visual disturbance, or proprioceptive disorders. A permanent disability in the form of imbalance without vertigo may occur. The patient should be made aware of alternate forms of therapy, both medical and surgical, including those surgical techniques that are designed to preserve residual hearing, such as selective vestibular nerve section or endolymphatic sac decompression, or those surgical procedures that may abate the vestibular symptoms without significant postoperative vestibular morbidity—for instance, round window labyrinthotomy or endolymphatic sac decompression.