2. Dunn LJ, Bradbury JT: Endocrine factors in endometrial carci- noma: a preliminary report. Am J Obstet Gynecol 97:465-471, 1967

2a. Florey CV: The use and interpretation of ponderal index and other weight-height ratios in epidemiological studies. J Chronic Dis 2393-103, 1970

3. Schoenberg BS, Christine BW, Wishnant JW: Nervous system neoplasms and primary malignancy of other sites. Neurology

4. Wynder EL, Gori GB: Contribution of the environment to cancer incidence: an epidemiologic exercise. J Natl Cancer Inst 582325- 832, 1977

5 . Wynder EL, Mabuchi K: Etiologic and preventive aspects of hu- man cancers. Prev Med 1:300-334, 1972

251705-712, 1975

Sudden Deafness in Chickenpox: A Case Report Rajan Bhandari, MD," and Gerald S. Steinman, M D

In a review of sudden deafness, Jaffe [4] reported 143 cases, 5 of which showed bilateral involvement and only 1 of which was attributed to chickenpox. The case report was not de- scribed, and it is unclear whether the hearing loss was unila- teral or bilateral, permanent or temporary. Standard text- books of pediatric neurology [ 1,9] and neurology [ 51 do not mention hearing loss as a complication of chickenpox.

A fourteen-month-old infant in previous good health de- veloped the characteristic papulovesicular rash of chickenpox accompanied by excessive upper respiratory symptoms, feed- ing difficulty, fever, vomiting, irritability, and lethargy. Five days later he developed gait ataxia, which progressed to marked swaying of the body, frequent falls, and a refusal to walk. The next day his mother noted the onset of auditory inattention, and the day after he was admitted to the hospital. Developmental milestones and language were appropriate for age. There was no family history of progressive neurolog- ical disease.

Physical examination upon admission revealed a playful infant who was in no acute distress if left alone. His general examination was normal except for healing crusted lesions consistent with chickenpox.

The neurological examination revealed a normal fundo- scopic appearance with good extraocular movements and pupillary responses. The infant did not react to clapping, calling his name, or loud bangs. Muscle strength and tone were normal. Rapid alternating movements and rapid fast movements were minimally slowed. While sitting, he demon- strated good posture without titubations. He was unable to stand without two-handed support and was quite ataxic when he walked. His muscle stretch reflexes were hyperactive but symmetrical and his toes were down-going on plantar stimu- lation. Sensory examination yielded normal responses to pin-

*Present address: Department of Neurology, Stanford University and Department of Neurology, Santa Clara Valley Medical Center, San Jose, CA 95128.

prick, light touch, and vibration, and proprioception was nor- mal.

Routine laboratory data revealed a mild microcytic hypo- chromic anemia. A head computed tomographic scan utiliz- ing contrast was normal. An EEG showed bilateral diffuse and excessive slowing without epileptiform activity. Spinal fluid analysis and culture were normal. Pure-tone audiometry revealed extensive bilateral hearing loss. Brainstem auditory responses could not be evoked using maximal stimuli of 93 dB and averaging 1500 clicks.

Over the following three weeks, the infant's coordination returned to ncrmal. Six months later he was still completely deaf as judged by absence of response to loud noise.

The nervous system complications of chickenpox are rela- tively infrequent and include cerebellar ataxia, aseptic menin- gitis, acute transverse myelitis, chickenpox-Reye syndrome, and, less commonly, aphasia, hemiplegia, and seventh cranial nerve palsy [ 5 ] .

Congenital deafness has occurred with rubella, mumps, rubeola, cytomegalovirus, and varicella-zoster virus 17, 81. In such cases deafness has been thought to occur secondary to endolymphatic labyrinthitis secondary to viremia or, perhaps, a perilymphatic infection C81. Alternate speculations are of a postinfectious allergic demyelination [2], although in a re- view of varicella cases, clear-cut evidence was lacking 131. Other causes of sudden, severe, at times complete (even per- manent) deafness include common cold, upper respiratory infection, meningitis, head trauma, hypertension, and, rarely, carbon monoxide poisoning 141.

It appears that this patient suffered bilateral complete deafness without vestibular involvement, as reflected by the absence of nystagmus and lack of lateralized ataxia. Hearing aids did not improve auditory evoked responses, although improvements have previously been reported [GI.

Department of Neurology Ohio State University Columbus, OH 4321 0

References 1. Bell WE, and McCormick WF: Neurologic infections in child-

hood. Philadelphia, Saunders, 1975, pp 217-218 2. Dutta SR, Barat D: Post infective allergic demyelination following

varicella. J Assoc Physicians India 25:577-580, 1977 3. Griffith JF, Salam MV, Adams RD: The nervous system disease

associated with varicella. Acta Neurol Scand 46279-300, 1970 4. Jaffe BF: Sudden deafness: an otologic emergency. Arch

Otolaryngol 86:81-86, 1967 5 . McKendall R, Klawans H: Nervous system complications of

varicella-zoster virus. In Vinken PJ, Bruyn G W (eds): Handbook of Clinical Neurology, Vol 30. Amsterdam, North Holland, 1978, pp 161-183

6. Rapin I , Graziani LJ: Auditory-evoked responses in normal, brain-damaged, and deaf infants. Neurology (Minneap) 17:881- 894, 1967

7. Siege1 M: Congenital malformations following chickenpox, measles, mumps, and hepatitis: results of a cohort study. JAMA 226:1521-1527, 1973

8. Strauss M, Gustave DL: Viral disease of the labyrinth. I. Review of the literature and discussion of the role of CMV in congenital deafness. Ann Otol Rhino1 Laryngol 82:577-583, 1973

9. Swaiman KF, Wright FS: The practice of pediatric neurology. St Louis, Mosby, 1975, pp 582-583

Notes and Letters 347