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Delayed-Onset Sensorineural Hearing Loss in a 3-Year-Old Survivor of Persistent Pulmonary Hypertension of the Newborn

Mark E. Hutchin, BS; Carol Gilmer, MS, CCC-A; Wendell G. Yarbrough, MD
Arch Otolaryngol Head Neck Surg. 2000;126(8):1014-1017. doi:10.1001/archotol.126.8.1014.
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Persistent pulmonary hypertension of the newborn (PPHN) and its conventional medical treatment are associated with sensorineural hearing loss, yet current recommendations for regular audiological evaluations of PPHN survivors are lacking. We report a case of delayed-onset, progressive sensorineural hearing loss in a 3-year-old patient with a history of PPHN and a normal auditory brainstem evoked response at 6 weeks of age. The relatively late detection of significant sensorineural hearing loss in this otherwise healthy 3-year-old illustrates the need for audiological evaluation at regular intervals in patients with a history of PPHN.

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Figure 1.

Normal auditory brainstem evoked response at 6 weeks of age demonstrating reproducible waveforms bilaterally at a stimulation level of 30 dB normal hearing level. Arrows indicate auditory brainstem evoked response wave V. No abnormality was detected at higher levels of stimulation.

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Figure 2.

Audiograms indicating moderate to severe bilateral sensorineural hearing loss. Comparison of the initial audiogram obtained at 2 years 11 months of age (left) and an audiogram obtained 6 months later (right) indicates progressive hearing loss. Type A tympanograms on both occasions and bone conduction testing confirmed the hearing loss as sensorineural.

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