Positive surgical indications for an only hearing ear were evaluated in order to improve patients' quality of life. Fifteen cases of surgery involving an only hearing ear over the past eight years were retrospectively reviewed. Of eight perforated chronic otitis media cases, seven underwent type one tympanoplasty and one underwent simple underlay myringoplasty regardless of otorrhoea at the time of surgery. Of six cholesteatoma cases, two received the canal wall up method and four received the canal wall down method. Ossiculoplasty was carefully performed in six cases. Hearing was improved in seven cases, whereas it remained unchanged in seven cases and deteriorated in one case. Of nine patients, two did not need a hearing aid after surgery. Five patients with severe combined hearing loss (>90 dB) were able to communicate with a hearing aid, alleviating their anxiety regarding hearing loss. Only hearing ears with chronic otitis media and cholesteatoma can be successfully treated by tympanoplasty with or without ossiculoplasty.

Diffuse infiltration of the meninges by metastatic carcinoma (meningeal carcinomastosis) is a potential complication of systemic malignancy. It may present with a variety of neurological symptoms as any aspect of the neuraxis can be affected. Often there is a history of pre-existing malignancy.

The authors describe a case with an initial presentation of sudden onset profound bilateral sensorineural hearing loss. The underlying pathology was found to be an occult breast carcinoma, a previously unreported finding.

The role of cerebrospinal fluid cytology and radio-imaging in diagnosis is discussed. All previously reported cases of sudden hearing loss and meningeal carcinomatosis are reviewed.

Branchio-oto-renal syndrome is a rare autosomal dominant condition characterized by hearing loss, branchial arch abnormalities and renal tract malformations. We present the first reported case of branchio-oto-renal syndrome associated with bilateral congenital cholesteatoma and ossicular chain abnormalities. The pathogenesis of this syndrome is described and the literature is reviewed.

Severe hearing impairment (SHI) is an impairment of hearing in which the air conduction mean over 0.5–4 kHz in the better hearing ear lies between 70 and 90 dB. Masking of bone and air conduction thresholds is seldom possible with SHI, leading to difficulties in assessing the pure tone audiogram whenever there is a potential air bone gap in either ear.

An audiological survey of 83 patients with severe hearing impairment was conducted. In 16 per cent of patients there was no possibility of a material air bone gap in either ear and no difficulty in interpreting the audiogram. In 23 (28 per cent), the audiogram was consistent with, but did not prove the presence of, an air bone gap in the poorer hearing ear. In 25 patients (30 per cent of 108) the better hearing ear could not be determined, and in 22 (27 per cent) a profound sensorineural impairment in the poorer hearing ear could not be excluded. The implications of this for the otologist are discussed.