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Monica G. Amatuzzi, MD; Clarinda Northrop; M. Charles Liberman, PhD; Aaron Thornton, PhD; Christopher Halpin, PhD; Barbara Herrmann, PhD; Luis E. Pinto, MD; Alberto Saenz, MD; Alfonso Carranza, MD; Roland D. Eavey, MD

Arch Otolaryngol Head Neck Surg. 2001;127:629-636.

Background  Deafness and handicapping sensorineural hearing impairment occur frequently in neonatal intensive care unit survivors for unknown reasons.

Patients and Methods  Hearing was tested early and repeatedly in neonatal intensive care unit patients with an auditory brainstem response (ABR) screener. The temporal bones of 15 nonsurvivors (30 ears) were fixed promptly (average, 5 hours) after death for histological evaluation.

Results  Among these patients, 12 failed the ABR screen bilaterally, 1 passed unilaterally, and 2 passed bilaterally. Cochlear histopathologic conditions that could contribute to hearing loss included bilateral selective outer hair cell loss in 2 patients, bilateral selective inner hair cell loss in 3 (all premature), and a combination of both outer and inner hair cell loss in 2. Other hair cell abnormalities were noted; the 2 infants who had passed the ABR screen demonstrated normal histological features. Neuronal counts were normal.

Conclusions  Auditory brainstem response failure among these neonatal intensive care unit infants who died was extremely common in part owing to an unexpected histological alteration, selective inner hair cell loss among premature newborns, that should be detectable uniquely by the ABR testing method. Additional histological patterns suggest more than one cause for neonatal intensive care unit hearing loss. Hair cell loss patterns seem frequently compatible with in utero damage.

From the Departments of Otolaryngology (Drs Amatuzzi, Liberman, and Eavey and Ms Northrop) and Audiology (Drs Thornton, Halpin, and Herrmann), Massachusetts Eye and Ear Infirmary, Boston; Department of Otology and Laryngology, Harvard Medical School, Boston, (Drs Liberman, Thornton, Halpin, Herrmann, and Eavey); Departments of Pediatrics (Drs Pinto and Saenz) and Pathology (Dr Carranza and Ms Northrop), Hospital Nacional de Niños, Escuela Autonomade Ciencias Medicas, Universidad Autonoma de Centro America, San Jose, Costa Rica; and Department of Otorhinolaryngology, Universidade de Sâo Paulo, Sâo Paulo, Brazil (Dr Amatuzzi).

Corresponding author: Roland D. Eavey, MD, Massachusetts Eye and Ear Infirmary, 243 Charles St, Boston, MA 02114.

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