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In discussing the prevalence of permanent congenital hearing loss (PCHL), it is important to consider definitional issues related to severity and type of hearing loss, the age of onset of that hearing loss, and whether the hearing loss is unilateral or bilateral loss (see slide #12). It is important to note that the numbers reported from the retrospective studies shown in slide #13 could have included children with late onset or acquired hearing losses.
Send Home Our method Usage examples Index Contact StatisticsWe do not evaluate or guarantee the accuracy of any content in this site. Hearing loss with craniofacial syndromes, and check out Hearing loss with craniofacial syndromes on Wikipedia, Youtube, Google News, Google Books, and Twitter on Digplanet. Individuals with Treacher Collins syndrome often have both cleft palate and hearing loss, in addition to other disabilities. The hearing loss most typical in patients with Stickler syndrome is a sensorineural hearing loss, indicating that the source of the deficit lies in the inner ear, the vestibulocochlear nerve, or the processing centers of the brain. Patients with Apert syndrome have a high occurrence of middle ear disease, otitis media and conductive hearing loss (Perterson-Fazone et al., 2001). A conductive hearing loss along with middle ear disease is most commonly seen in patients with Pfeiffer syndrome; although, there have been reports of mixed hearing loss as well. Conductive hearing loss has been reported by many with ectrodactyly-ectodermal dysplasia-cleft (EEC) syndrome in association with a cleft palate (Perterson-Falzone, 2001). In Saethre-Chotzen syndrome the ears may be low set, posteriorly rotated, have other minor anomalies, and there may be a presence of a conductive hearing loss or a mixed hearing loss (Perterson-Falszone, 2001). About 70% of individuals with velocardiofacial syndrome (VCFS) have minor auricular malformations, or malformations of the ear. Individuals with hemifacial microsomia, also called oculoauriculo-vertebral spectrum, often have ear malformations.
Individuals with Nager syndrome typically have the malformations of the auricle, external auditory canal, and middle ear, including the ossicles. Cleft Palate-Craniofacial Journal Online for scholarly, peer-reviewed articles on topics related to clefting.
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Consider first the results of 11 different population-based studies designed to determine the number of children with bilateral PCHL in population-based cohorts ranging in size from 10,000 to over 4 million children. If children with unilateral losses had been included, the prevalence would increase by one-third to one-half as shown in slide #14. Many people assume that there are about as many young children with acquired losses as with congenital losses.

Many of these multianomaly disorders involve structural malformations of the outer or middle ear, making a significant hearing loss highly likely. Hearing loss is often secondary to absent, small, or unusually formed ears (microtia), and commonly results from malformations of the middle ear. One study showed an average of 83% hearing loss in PRS, compared to 60% in cleft individuals without PRS (Handzic et al., 1995).
Conductive hearing loss is frequently seen in this population due to almost constant middle ear disease (Gould et al., 1982).
Literature has suggested that persons with Crouzon syndrome typically have conductive hearing loss caused by middle ear effusion (or fluid in the middle ear) and perforation to ossicular fixation (ossicles), intratympanic bony masses (tympanic membrane), ossicular anomalies, and closure of the oval window.
The hearing loss is most typically caused by stenosis or atresia of the auditory canal, middle ear hypoplasia, and ossicular hypoplasia (Vallino-Napoli, 1996). These malformations can be in the form of preauricular ear pits, complete absence of the auricle, stenosis or atresia of the external auditory canal, ossicular malformations, middle ear deformities, and incomplete pneumatization of the temporal bone. Not surprisingly, as shown in slide #13, the prevalence of bilateral PCHL is substantially higher when more lenient definitions are used. Based on these data, we should expect to find 3 to 4 children per thousand with PCHL from well-run, hospital-based, UNHS programs.
However, states with long-established UNHS programs, where tens of thousands of infants have now been screened, are finding very few children with acquired losses.
Researchers have found that most patients with Treacher Collins syndrome have symmetric external ear canal abnormalities and symmetrically dysmorphic or absent ossicles in the middle ear space. Similarly, PRS individuals typically exhibit conductive, bilateral hearing losses that are greater in degree than in cleft individuals without PRS. Furthermore inner ear anomalies have been described in Apert syndrome, such as dilatation of the vestibule, dysplastic semicircular canals and cochlear malformations (Zhou G et al.
Patients with a sensorineural hearing loss have also been reported, but are less likely to occur.
In addition to external malformations, individuals with VCFS are more vulnerable to otitis media because of the presence of a cleft or other form of velopharyngeal inadequacy.
When children are required to have a 50 dB bilateral loss before they are considered to be hearing impaired, the prevalence is about one per thousand. For example, in Rhode Island, Hawaii, Colorado, and Utah, hundreds of children have now been identified with PCHL. The hearing loss associated with VCFS is conductive when otitis media is present (Peterson-Falzone et al., 2001).

However, when children with 30 dB bilateral losses are included, the prevalence increases to about 2.5 per thousand. Many of those programs have been operating long enough that there are thousands of 7, 8, and 9 year olds who were screened as infants. Most patients show a moderate hearing impairment or greater, and the type of loss is generally a conductive hearing loss.
Accordingly, management by ear tubes (myringotomy tubes) is often effective and may restore normal levels of hearing (Handzic et al., 1995).
This mildly progressive sensorineural loss, or more significant losses (associated with Types II and III Stickler syndrome) is present in about 80% of patients with Stickler syndrome. In addition to ear malformations, a conductive hearing loss can be present, typically ranging from mild to severe.
If acquired hearing loss were as frequent as is often assumed, we should now be discovering dozens of children with late onset losses as they enter school. Patients with Treacher Collins syndrome exhibit hearing losses similar to those of patients with malformed or missing ossicles (Pron et al., 1993). However, other patients are also susceptible to conductive losses, similar to nonsyndromic cleft patients (Peterson-Falzone et al., 2001).
Of individuals with VCFS who have a hearing loss, only 11% had a sensorineural loss and 5% a mixed loss (Reyes et al., 1999). For this reason, early detection and treatment for middle ear disease is crucial in this population. The emerging hypothesis (see slide #16) is that many of the children, who were previously thought to have late onset losses, were really children with congenital mild or moderate progressive PCHL.
As infants, they had enough hearing that they began to babble, acquire some speech, startle, or turn to loud noises.
There is no correlation between the severity of dysmorphic features and the degree of hearing loss, meaning individuals with mild malformations can have severely impaired hearing. By the time they were 3, 4, or 5 years old, however, it was clear that their speech was substantially delayed, and as a result, hearing loss was diagnosed. Because they were babbling and turning to noises as an infant, it was assumed that it was an acquired loss, when in fact many of these could have been mild or moderate progressive congenital losses.

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