We report the first case in the world literature of deliberate ablation of vestibular function in unilateral Meniere's disease with aminoglycoside eardrops (Garasone).

These findings give conclusive proof that Garasone eardrops, and by implication, all topical aminoglycoside ear drops can be vestibulotoxic in the presence of a tympanic membrane defect. The lower concentration of gentamicin in Garasone, in comparison to the standard intratympanic preparations for chemical ablation of vestibular function, may also offer a reduction in the potential risk of cochleotoxocity.

The surgical management of Menière's disease presents a difficult and controversial problem and includes various procedures ranging from ‘placebo operations’ to vestibular nerve section. The situation is even more complex in the case where the only hearing ear is affected. We present a case of severe debilitating vertigo due to Menière's disease that was treated by chemical labyrinthectomy and cochlear implantation. This unique management strategy allowed restoration of useful hearing and relief of vertigo.

Records are available on 27 patients who had vestibular nerve section between 1975 and 1987 giving a follow-up time of 10 to 22 years with a mean of 16 years. Episodic vertigo was fully controlled in 26 patients, one needing a labyrinthectomy 18 months later.

In addition to the patient who had labyrinthectomy, one patient had immediate post-operative profound hearing loss so that long-term follow-up of hearing was possible in 25 patients. After six months there was an overall average improvement in hearing of 1 dB. Thereafter there were average deterioration of 7 dB at two years, 15 dB at 10 years, 23 dB at 15 years and 29 dB at 20 years. This deterioration was more marked in those who had better hearing at the time of surgery, with all 10 whose hearing was classified as good deteriorating to poor by 15 years. At 10 years hearing deteriorated by 25 dB in those with good hearing and by 7 dB in those with poor hearing. In 20 per cent of patients there was audiological evidence of eventual involvement of the other ear.

Tinnitus became worse in five patients but was a major problem in only one patient. One patient suffered a facial paralysis with partial recovery and, in addition, in one the frontal branch was divided in the incision.

In 1861 Prosper Menière separated patients with episodic vertigo, hearing loss and tinnitus from a group previously described as having apoplectiform cerebral congestion. He suggested the cause was disease within the semicircular canals (Menière, 1861). Over the years it becoame apparent that within this group there were a number of patients with characteristic signs and symptoms and in 1938 a pathological correlate was found in the form of endolympatic hydrops. Descriptions scuh as Menière's ‘Menière's ‘disease’, Menière's ‘syndrome’ and Menière' ‘symptom complex’ led to a confusing array of terms for this condition and monotoring of treatment results became difficult. In response to this in 1972 the American Academy of Ophthalmology and Otolaryngology and Otolaryngoly Committee on Hearing and Equillibrium published a clear definition of Menière's disease and criteria for the reporting of treatment results, it was updated in 1985 and again in 1995. We described the changes that have taken place as the definition of Menière's disease has evolved.

Between April 1990 and June 1996, the extraosseous portion of endolymphatic sac has been removed from the affected ear in 77 patients suffering from Menière's disease. Removal of the extraosseous part of the endolymphatic sac without any drainage procedure did not increase the frequency or severity of the attacks of vertigo in any patient. The results of 43 patients with unilateral disease who had a follow-up period of two years are presented. Only eight of the patients had more than two recurrent attacks of vertigo lasting over two minutes within the two years after the surgery, and in three of these patients the severity of the attacks was greatly reduced. In 56 per cent of the operated ears, the hearing deteriorated at least 10 dBHL across five audiometric frequencies (250 Hz, 500 Hz. 1 kHz. 2 kHz and 3 kHz). To the author, endolymphatic sac removal appeared to provide better relief from vertigo than a simple drainage procedure with less tendency for recurrence several months or years after the initial surgery. At present, the statistical analysis of the results shows no significant difference between removal of the extraosseous portion of the endolymphatic sac and the ‘so called’ endolymphatic sac drainage procedures.

Vestibular neurectomy is an effective procedure in the management of vertigo due to active labyrinthine disease. Various approaches have been developed for selectively sectioning the vestibular nerve, in order to preserve serviceable hearing and avoid facial nerve injury.

In patients who have a mastoid cavity, from previous surgery for chronic otitis media, the approach to the vestibular nerve has to be modified. Considerations taken into account are cavity infection, hearing status, and the presence of associated loud tinnitus.

Vestibular or vestibulo-cochlear nerve section has been undertaken, by the senior author, in eight patients with a mastoid cavity from previous surgery for chronic otitis media. Translabyrinthine, retrosigmoid and middle fossa approaches have all been used; strategies for selection of each specific technique are considered, and the aetiology of post-chronic suppurative otitis media (CSOM) peripheral vestibular disease discussed.

A labyrinthectomy is known to relieve vertigo successfully in the majority of patients who suffer from Menière's disease and have non-serviceable hearing in the affected ear. It is assumed that the procedure reduces disability, helps the patient to return to work and improves the quality of life. Eighteen patients who underwent a transmastoid drill-out labyrinthectomy between 1980 and 1990 were interviewed and an attempt was made to evaluate the success of the operation in accordance with the guidelines set out by the AAO-HNS 1985. In the present series it was noted that although vertigo was relieved in 89 per cent of patients after labyrinthectomy, only 50 per cent of them returned to work. In this study, the age and occupation of the patient at the time of surgery and the relief of vertigo did not accurately predict whether or not a patient returned to work.

The aetiology of Menière's disease (MD) remains obscure, but is likely to be multifactorial, one of the factors being a genetic predisposition. Forty-one families with more than one living member with MD were ascertained and affected and normal relations examined. Blood was collected and DNA extracted and stored. In these families the mode of inheritance is autosomal dominant, the penetrance of the mutation being about 60 per cent. Some of the family members exhibit a partial syndrome, vestibular symptoms predominating. Sporadic and familial cases exhibit the same clinical features. The striking finding is the phenomenon of anticipation, whereby with successive generations there is an earlier age of onset and a tendency to more severe manifestation. The inference, considering that the cells which regulate endolymph are of neuroectodermal origin, is that, like other neurodegenerative disorders which show anticipation, MD manifestation is likely to be related to trinucloetide expansion within a gene.

Many procedures have been devised to deal with intractable vertigo and conserve hearing, but despite this selective vestibular nerve section remains by far the most effective treatment.

A series of 14 patients who underwent posterior fossa vestibular neurectomy is reported. The results are reported for vertigo control, hearing and tinnitus. All of the patients achieved vertigo control according to the AAOO (1972) reporting system. A simple and reliable system for the classification of the disability in these patients has yet to be devised. This problem is addressed and a disability grading system proposed, and discussed.