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Are the consequences of neonatal hearing loss serious enough that we need to be concerned about them in the first place? Slide #47 shows data from an annual survey done each year by Gallaudet University showing that children with severe profound bilateral losses suffer from substantial deficits in reading comprehension. Although virtually everyone agrees that severe profound bilateral hearing loss has substantial negative consequences for all aspects of academic performance, the consequences of mild bilateral or unilateral hearing loss are less well known. The data in slide #50 come from the Greater Boston Otitis Media Study Group, where 194 children were followed prospectively for 7 years.
Deafness in young animals is a problem affecting over 60 breeds of dogs as well as white-coated cats. Although it is not usually difficult to recognise a bilaterally deaf pet, it can be very difficult to detect unilateral deafness. Indications for performing the BAER test include early litter screening in breeds where deafness is a problem, detection of acoustic neuromas and posterior fossa tumours and  intraoperative monitoring during brain surgery. Hearing loss affects 1 to 3 infants per 1000 births and is the most common congenital sensory disorder. Babies learn language early by experiencing the world around them through the use of all their senses. If you suspect that your child has a hearing loss, contact us at 972-566-7359 to schedule an appointment to test your childa€™s hearing.
NIDCD CMV Grant, UAB Multi-site investigation, 7 sites 100,000 newborns to be screened for congenital CMV infection Link newborn hearing results Evaluate methods for CMV screening Saliva vs. Screen at least 100,000 newborns for CMV infection who currently undergo newborn hearing screening Audiological follow-up of all CMV positive infants Compare the accuracy of two diagnostic methods for CMV screening (traditional rapid saliva cell culture vs.
Cultural Diversity Project informational materials will be provided in English and Spanish for parents and at understandable reading levels.
The triad of short neck, low posterior hairline and severe restriction of cervical motion is a classic definition of Klippel-Feil syndrome. A 27 year old male, unmarried from rural background was admitted with complaints of progressive generalized weakness, dyspnea on exertion, puffiness of face for one month and severe anorexia, hiccoughs and decreased urine output for about a week. KFS occurs in a heterogeneous group of patients unified only by the presence of a congenital defect in the formation or segmentation of the cervical spine.
In conclusion, there is high incidence of congenital anomalies of the genito-urinary tract in patients with KFS so they should be made aware of the potential complications. Grise P, Lefort J, Dewald M, Mitrofanoff P, Borde J.Urinary malformations in synostoses of the cervical vertebrae (Klippel-Feil syndrome).
Fortunately, there is a wealth of data on this question, and only three very brief examples will be given related to children with severe profound bilateral losses, children with mild bilateral and unilateral losses, and children with fluctuating conductive losses (slide #46).

There are, however, a number of studies demonstrating that even mild or unilateral sensorineural hearing loss has substantial negative consequences. Although virtually all newborn hearing screening programs focus only on identifying children with permanent congenital hearing loss, some children with fluctuating conductive losses are often identified.
Each time the child visited his or her primary- care physician, data were collected about episodes of otitis media (there were an average of 7 visits per year during the birth to 3-year-old period). Although not a serious health issue in itself, deafness can be a handicap for pets and difficult for their owners.
Traditional methods such as clapping or rattling keys are subjective and dogs, in particular, can quickly learn to compensate for unilateral deafness by picking up on vibrations, smells and changing air currents. In young dogs carrying the piebald or merle genes sensorineural deafness is a result of the death of the cochlear hair cells following the degeneration of the stria vascularis in the cochlear duct. Tiny recording needles are inserted just under the skin on the head and the mastoid processes.
Your childa€™s hearing loss does not hinder him or her from being able to enjoy all the sounds of life if amplification and support are provided early. Klippel-Feil syndrome (KFS) is characterized by congenital fusion of two or more cervical vertebrae and is believed to result from faulty segmentation along the embryoa€™s developing axis during the second to eighth week of gestation.
When his history was traced back, he had short neck, slight facial dysmorphism and restriction of neck movements since childhood. On the basis of history, clinical examination, laboratory investigations and imaging he was diagnosed to have KFS (Figures 1 and 2) with end-stage renal disease and unilateral agenesis of left kidney (Figure 3). Brain stem anomalies, congenital cervical stenosis, adrenal aplasia, ptosis, lateral rectus muscle paralysis, facial nerve paralysis, syndactilia, and diffuse or focal hypoplasia in the upper extremities may also be seen.
Existing embryologic data suggest that the blastoma of the cervical vertebrae, scapulae, and the genitourinary system have an intimate spatial relationship at the end of the 4th or beginning of the 5th week of fetal life. Detection of KFS should stimulate careful search for other associated systemic abnormalities.
That gap continues to widen over time, with the average deaf child or youth never exceeding a grade equivalent of 3 years, in spite of the fact that most of them are enrolled in educational programs specifically designed for deaf students. Slide #48 summarizes information about five different students in which a sample of children with unilateral hearing loss were compared to similar children with normal hearing, and measures were taken about their performance in math, language, and social functioning.
The reason for presenting this information is to emphasize the point that even mild fluctuating hearing losses have an important negative effect on developmental outcomes.
Data were also collected periodically on a wide variety of measures related to intellectual ability, cognitive functioning, and language competency by trained diagnosticians who were uninformed about the purposes of the study. Deafness can affect either one ear (unilateral deafness) or both ears (bilateral deafness). These record the brainwaves, as the potential moves along the acoustic nerve and a wave form is generated on the screen.
This number increases when fluctuating hearing loss from ear infections, high frequency hearing loss and unilateral hearing loss are included.

With early diagnosis and intervention, children will have access to speech, language, and important sounds. There had been no major trauma, surgery, systemic illness or prolonged treatment in the past.
Type 1: Cervical spine fusion in which elements of many vertebrae are incorporated into a single block. An alteration in this region can affect the cervical vertebrae and scapulae directly, and the genitourologic changes are mediated indirectly through the inductive capacity of the pronephric duct.
As shown in slide #49, children with unilateral hearing loss lagged substantially behind their peers regardless of the measure used, whether math, language, or social functioning. Results at 7 years of age for two groups of children are reported here: those with less than 30 days of otitis media over the 3-year period compared to those with 130 or more days of otitis media over the same period.
With hearing loss, your child will have difficulty dealing with challenges and opportunities at home and in school. The anatomic and clinical expressions of this syndrome vary widely, ranging from mild cosmetic deformity to severe disability and end stage target organ diseases such as renal failure. Type 2: Cervical spine fusion in which there is failure of complete segmentation at only one or two cervical levels and may include an occipito-atlantal fusion. The high incidence of genitourinary anomalies with Klippel-Feil syndrome has been described in the literature.1,2 Grise et al3 have demonstrated 52% incidence in their series of 35 cases with Klippel-Feil syndrome. Even though the sample sizes are quite small in each of the studies, matching was generally done quite well and the results are very consistent. After statistically adjusting the results for a variety of demographic and socio-economic status variables, children with fewer episodes of otitis media did substantially better than their peers on all measures.
Hereby, we report a patient with KFS who had unilateral renal agenesis and presented with end stage renal disease. Type 3: Type 1 or type 2 fusion with co-existing segmentation errors in the lower dorsal or lumbar spine. The difference averaged about one-half standard deviation, which is equivalent to more than a year's worth of development in reading or math achievement. There was severe pallor, anasarca, limitation of neck extension and rotation, low hair line, scoliosis and Sprengela€™s deformity of the left scapula.
Most of these patients remain asymptomatic but risk of renal failure always exists.3 The common genito-urinary abnormalities include unilateral renal agenesis, malrotation of the kidney, renal pelvis and ureteral duplication, renal ectopia, renal dysgenesis, hypospadias, cryptorchidism, vaginal agenesis and bicornuate uterus. The cause of renal dysfunction in the single kidney could not be established in our patient but presence of anatomic defect on the other side as well might have resulted in renal failure. Abdomen examination revealed presence of ascites and auscultation of chest showed presence of few basal crepitations.

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