Auditory agnosia due to long-term severe hydrocephalus caused by spina bifida – specific auditory pathway versus nonspecific auditory pathway

Abstract

A 27-year-old female showed auditory agnosia after long-term severe hydrocephalus due to congenital spina bifida. After years of hydrocephalus, she gradually suffered from hearing loss in her right ear at 19 years of age, followed by her left ear. During the time when she retained some ability to hear, she experienced severe difficulty in distinguishing verbal, environmental, and musical instrumental sounds. However, her auditory brainstem response and distortion product otoacoustic emissions were largely intact in the left ear. Her bilateral auditory cortices were preserved, as shown by neuroimaging, whereas her auditory radiations were severely damaged owing to progressive hydrocephalus. Although she had a complete bilateral hearing loss, she felt great pleasure when exposed to music. After years of self-training to read lips, she regained fluent ability to communicate. Clinical manifestations of this patient indicate that auditory agnosia can occur after long-term hydrocephalus due to spina bifida; the secondary auditory pathway may play a role in both auditory perception and hearing rehabilitation.

Keywords::

• Subcortical hearing loss
• music
• auditory perception
• hearing rehabilitation

Acknowledgments

We thank speech pathologists at the national hospital for handicapped children in Tokyo for their audiological examinations. This work was supported in part by a research grant from the Society for the Promotion of International Otorhinolaryngology (Japan), by a scholarship from Takeda Science Foundation (Japan), and by a school president grant from Xi'an Jiaotong University (xjj 2008019, China). This is a work of the National Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.