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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. Audiometry results from the 2012 and 2013 Canadian Health Measures Survey (CHMS) indicate that 20% of adults aged 19 to 79 years had at least mild hearing loss in at least one ear (Chart 1). The survey assessed hearing loss as either unilateral (occurring in only one ear) or bilateral (occurring in both ears). Audiometry results indicate that 5% of children and youth aged 6 to 18 years had hearing loss that was considered mild or worse (Chart 2).
The majority of Canadians with measured hearing loss were not aware they had any hearing problems (Chart 3). 42% of Canadians aged 16 to 79 years have worked or currently work in an environment where it is required to speak in a raised voice to communicate with someone standing an arm’s length away. 51% of Canadians aged 3 to 79 years have used earbuds or headphones to listen to music, movies or other types of audio in the last 12 months. 41% of Canadians aged 3 to 79 have experienced tinnitus (hissing, buzzing, ringing, rushing or roaring sounds in the ears). Sensorineural hearing loss is the most common type of permanent hearing loss and occurs when there is damage to the inner ear or the nerve pathways from the ear to the brain.
Audiometric tests were administered as part of the Canadian Health Measures Survey to assess sensorineural hearing loss in children and adults aged 6 to 79. Otoscopy, tympanometry and distortion product otoacoustic emissions (DPOAE) tests were also conducted on respondents aged 3 to 79. Additional Canadian Health Measures Survey data on this topic are available in CANSIM table 117-0022. If you are really benefited , Please share it with your friends on Facebook by clicking Fshare button.
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. Tinnitus is identified more frequently in white individuals and the prevalence of tinnitus is almost twice as frequent in the South as in the Northeast. Hearing loss was unilateral in 48% and bilateral in 52% of adults with at least mild hearing loss (data not shown). However, results from the otoacoustic emissions testing indicate that about 7% of children aged 3 to 5 had results consistent with conductive hearing loss (data not shown, see About hearing). About 70% of adults with measured hearing loss did not report any diagnosis by a health care professional, while the same occurred in 83% of children and youth.
One-third of those individuals regularly listened at a volume that was at or above three quarters of the maximum volume.
Tympanometry measures the mobility of the ear drum and the pressure of the middle ear system. 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. Adults aged 60 to 79 years were significantly more likely to have hearing loss (47%) compared with younger adults aged 40 to 59 years (16%) and 19 to 39 years (7%). Among children and youth with hearing loss, the majority (90%) had hearing loss in only one ear (unilateral, data not shown). Males and females were similar in that 70% of males and 72% of females with measured hearing loss did not report having been diagnosed with a hearing loss. 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. Age related hearing loss is called presbycusis, and is prevalent in the elderly population.

About 2 to 3 out of every 1,000 children in the United States are born deaf or hard-of-hearing. Males (25%) were significantly more likely to have hearing loss compared with females (14%) (Chart 1). Nearly 2 in 10 adults aged 19 to 39 with hearing loss had bilateral loss compared with 5 in 10 of those aged 40 to 59 and 7 in 10 of those aged 60 to 79 (data not shown). 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.
This form of hearing loss can occur independently or in conjunction with sensorineural hearing loss. 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. In the United States, roughly 23,000 adults and 15,500 children have received them.Approximately 4,000 new cases of sudden deafness occur each year in the United States. 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. Another 45,500 are newly diagnosed each year.Approximately 3 to 6 percent of all deaf children and perhaps another 3 to 6 percent of hard-of-hearing children have Usher syndrome.
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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