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Chapter 8: Hearing and Ear Abnormalities in FA 169 was documented in the remaining 13 ears. The majority of hearing loss was mild, and the most common types of hearing losses were conductive hearing loss (9 cases, 65%), followed by sensorineural hearing loss (3 cases, 21%) and mixed hearing loss (2 cases, 14%). These findings suggest that over half of individuals with FA have a mild conductive hearing loss, probably due to an abnormally developed eardrum and/or ossicles in the middle ear space. Figure 2: Normal eardrum Abnormal eardrum in FA patient
170 <strong>Fanconi</strong> <strong>Anemia</strong>: Guidelines for Diagnosis and Management In summary, hearing loss, mostly conductive, is present in greater than 50% of our FA cohort ears. Congenital abnormalities in the tympanic membrane and middle ear ossicles were found in 67%. This incidence of hearing loss and congenital ear malformation is much higher than previously reported. This study suggests that these features characteristic of FA can occur even when hearing is normal or slightly reduced. Consequences of Hearing Loss Children use their hearing to develop speech, language, communication skills, and to facilitate learning. Children with a mild hearing loss (25-40 dB HL) have difficulty hearing faint and distant speech. Vowel sounds can be heard clearly, but voiceless consonants (e.g., “s,” “f,” and “th”) can be difficult to hear. School-age students with mild hearing loss have difficulty when functioning in a regular classroom with normally present background noises. 3 Children with moderate hearing loss (41-55 dB HL) miss much of normal conversation. These children may have difficulty learning vocabulary, grammar, and other aspects of verbal communication. Even children with unilateral hearing loss (one ear with normal hearing and other other with at least a mild permanent hearing loss) can be affected. If listening takes place in a consistently noisy environment, their academic potential and social interaction can be compromised. Early Identification and Intervention for Hearing-Impaired Children Once hearing loss is identified or suspected, the child should undergo comprehensive medical ear and audiologic evaluation. The earlier the hearing loss is identified and intervention initiated, the less serious the
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Chapter 4 Gastrointestinal, Hepatic
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Chapter 12 Novel Treatment Options
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Chapter 13 Head and Neck Squamous C
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Chapter 14 The Adult Fanconi Anemia
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