LaVonne Bergstrom

Hearing Loss in Renal Disease: Clinical and Pathological Studies

Ninety-one of 224 chronic renal patients, most of them hemodialysis and/or transplant patients, had sensorineural loss: 11% noise exposure; 7% genetic; 22% due to multiple factors, including ototoxicity; 41% with hearing loss and ototoxic drug exposure, (but one-fourth had had insufficient drugs to cause hearing loss) and 11% were of unknown etiology. Hearing loss and normal hearing patients exposed to ototoxicity or multiple factors were similar in all parameters but hearing loss. Ten temporal bone cases are described, two from patients with hearing loss of unknown origin. One showed cochlear hydrops, fibrous tissue proliferation in cochlear perilymphatic spaces, Cortis organ degeneration, displaced tectorial membrane and probable metastatic calcification in the stria vascularis. The other case showed hair cell loss. Three patients had had ototoxic drugs; one bad hair cell loss and tectorial membrane abnormalities; one had hair cell loss and metastatic strial calcification; one was normal. One patient had pus in both internal auditory canals. Another bad otosclerotic focus without stapes fixation. Three were normal. We do not yet recognize pathology typical of hearing loss of severe renal disease. Findings seem to fall into two broad categories: those of known entities and those of obscure etiology.

VII Rhinocerebral and Otologic Mucormycosis

CASE 1. DVAH #503-28-91-52 entered the Denver Veterans Administration Hospital in October, 1968 with a ten-day history of swelling, diplopia and considerable loss of vision in the right eye. The patient had been a known diabetic for four years. Disregarding his physicians advice, he had discontinued his insulin several months previously. Past history was negative for ocular, nasal or sinus disease. His family had noted personality changes of irritability and hostility in the weeks preceding admission.

Syndromes associated with congenital facial paralysis.

Thirty-five of 1,488 pediatric otologic cases had congenital facial nerve weakness. A cause was generally not found, but two probably had nuclear dysgenesis; one may have had an intracanalicular lesion; two cases resulted from teratogens, one from poor intrauterine environment, and three from genetic complications. Five had total unilateral paralysis; one had bilateral palsy. Frequent associated anomalies were microtia-atresia, hemifacial microsomia, facial clefts, Moebius syndrome, and congenital conductive sensorineural loss.

Sudden deafness and facial palsy from metastatic bronchogenic carcinoma.

A 52-year-old man developed sudden total bilateral deafness, and unilateral facial palsy, without other symptoms and findings. He died two months later of of bronchogenic carcinoma metastatic to dura, brainstem, pons, carebellopontine angle, cerebellum and cranial nerves III, VI, VII and VIII. There was bilateral internal auditory canal erosion. Tumour replaced right facial, acoustic and vestibular nerves. Tumour infiltrated spiral ganglion, cochlear nerve, cochlear aqueduct, and destroyed nearly all facial nerve fibres to the level of the stapedius muscle. No tumour cells were found on the left, but few fibres of facial, acoustic and vestibular nerves survived. Both ears showed some cochlear outer hair cell destruction. Metastatic tumour to temporal bone or dura should be considered when loss of peripheral VIIth or VIIIth nerve function occurs.

Pathological changes in congenital deafness.

The temporal bones of four infants with varying systemic and ear pathologies are described and discussed with reference to the probable effect on hearing and embryogenesis.

Pendred's Syndrome with Atypical Features:

One of seven cases of Pendreds syndrome studied had mixed hearing loss, bilateral congenital stapes foot-plate fixation, surgically proven unilateral perilymph fistula and delayed fluctuating hearing loss in the unoperated ear. A unifying hypothesis is proposed to link these diversified findings.

Clinical Pediatric Otolaryngology

This book has seven sections. The first deals with general areas of examination; anesthesiology (that chapter alone worth the price of the book); birth defects and genetic counseling; allergic diseases; and ear, nose, and throat manifestations of systemic disease. That chapter ends with useful brief lists of diseases that produce coagulopathies, head and neck infections, hearing loss, and mucosal ulcers. Developmental and anatomic chapters addressing specific disorders and their importance lead most sections. At the end of most chapters there are decision trees or algorithms that are quite useful. Chapters 19 through 22 are valuable for pediatric and general otolaryngologists. The authors discuss referral guidelines, speech disorders, auditory rehabilitation of the hearing-impaired child, and management of voice disorders, including stuttering. Section four deals with oropharyngeal and dentofacial development and disorders. The section in Chapter 23, on swallowing and sucking, is almost poetic, but informative. Pashleys chapter on cleft lip and