- Category: Diseases of the ear, nose and throat
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Hearing loss is considered to be the most prevalent congenital abnormality in newborns and is more than twice as prevalent as other conditions that are screened for at birth, such as sickle cell disease, hypothyroidism, phynilketonuria, and galactosaemia (Finitzo & Crumley, 1999). It is one of the most common sensory disorders and is the consequence of sensorineural and/or conductive malfunctions of the ear. The impairment may occur during or shortly after birth (congenital or early onset or may be late onset) caused post natal by genetically factors, trauma or disease. Hearing loss may be pre-lingual (i.e., occurring prior to speech and language acquisition) or post-lingual (i.e., occurring after the acquisition of speech and language).
Since hearing loss in infants is silent and hidden, great emphasis is placed on the importance of early detection, reliable diagnosis, and timely intervention (Spivak et al., 2000). Even children who have mild or unilateral permanent hearing loss may experience difficulties with speech understanding, especially in a noisy environment, as well as problems with educational and psycho-social development (Bess et al., 1988; Culbertson & Gilbert 1996). Children with hearing loss frequently experience speech-language deficits and exhibit lower academic achievement and poorer social-emotional development than their peers with normal hearing.
The period from birth to 3-5 years is often considered as the "critical period" for the development of normal speech and language. Normal hearing in the first six months of life is also considered critical for normal speech and language skills. Hence, early identification and appropriate intervention within the first six months of life have been demonstrated to prevent or reduce many of the adverse consequences and to facilitate language acquisition (Yowhinaga-Itano et al., 1998). Consequently, in developed countries with a high standard of health care, primary services include the early detection of congenital hearing loss and the initiation of auditory habilitation before six months of age.
The prevalence of congenital and early-onset hearing loss in most developed countries is estimated to range between 2-4 infants with moderate-severe hearing loss in every 1000 births. In contrast, only limited information is available on developing regions, including the Middle East especially in the Arab countries, where the prevalence is estimated to be markedly higher than in Israel or European and North American countries (Attias et al., 2006). In developing countries, more than 10 infants in every 1000 births are estimated to be affected by a severe profound hearing loss. Of the 62 million deaf children younger than 15 years old worldwide, two-thirds reside in developing countries (Smith, 2003).
Presbycusis (age-related hearing loss) is the loss of hearing that gradually occurs in most individuals as they grow older. Hearing loss is a common disorder associated with aging and is ranked as the third most prevalent chronic condition in elderly people after hypertension and arthritis. Its prevalence and severity increase with age, rising from about 30-35 percent of adults aged 65 and older to an estimated 40-50 percent of adults aged 75 and older (Cruikshanks et al., 1998). The loss associated with presbycusis is usually greater for high-pitched sounds. For example, it may be difficult for someone to hear the nearby chirping of a bird or the ringing of a telephone, and it is most difficult to understand speech in a noisy background. However, the same person may be able to clearly hear the low-pitched sound of a truck rumbling down the street. Presbycusis most often occurs in both ears, affecting them equally. Because the process of loss is gradual, people who have presbycusis may not realize that their hearing is diminishing.
There are many causes of presbycusis, though it is most commonly the result of changes in the inner ear as a person ages. It can also stem from changes in the middle ear or from complex changes along the nerve pathways leading to the brain.
The negative impact of hearing loss on older adults is significant (LaForge et al., 1992). Hearing loss is associated with depression, social isolation, poor self-esteem, and functional disability (Mulrow et al., 1990a), particularly for those suffering from hearing impairment who have not yet been evaluated or treated for hearing loss.
Hearing impairment is a broad term that refers to hearing losses of varying degrees, ranging from hard-of-hearing to total deafness. As the general population continues to age, the prevalence of hearing impairment can be expected to increase. Since the use of hearing aids or surgical intervention to improve hearing loss has been shown to have a positive impact on quality of life (Mulrow et al., 1990b, 1992; Weinstein, 1991), more screening programs for elderly adults should be established, followed by appropriate referral to audiologists based on individual needs. Without early diagnosis and treatment of hearing impairment, quality of life and functional status are likely to decline in the aging population.
The major challenge facing people with hearing impairment is communication. Hearing-impaired persons vary widely in their communication skills. Among the conditions that affect the development of communication skills by persons with hearing impairments are personality, intelligence, nature and degree of deafness, degree and type of residual hearing, degree of benefit derived from amplification by hearing aid, family environment, and age of onset. Age of onset plays a crucial role in the development of language. Persons with pre-lingual hearing loss (present at birth or occurring before the acquisition of language and the development of speech patterns) are more functionally disabled than those who lose some degree of hearing after the acquisition of language and speech.
When describing hearing impairment, three attributes are considered:
1. Type of hearing loss (part of the hearing mechanism that is affected).
2. Degree of hearing loss (range and volume of sounds that are not heard).
3. Configuration (range of pitches or frequencies at which the loss has occurred).
Types of hearing loss
A hearing loss can be classified as a conductive, sensory, neural, or mixed hearing loss, based on the anatomic location of the problem (site of lesion, i.e., middle or inner ear). A hearing loss may also be labeled as unilateral or bilateral, depending on whether the loss is in one (unilateral) or both (bilateral) ears. The degree of loss might be the same in both ears (symmetrical hearing loss) or it could be different for each ear (asymmetrical hearing loss).
Conductive hearing loss is characterized by an obstruction to air conduction that prevents the proper transmission of sound waves through the external auditory canal and/or the middle ear. It is marked by an almost equal loss of all frequencies. The auricle (pinna), external acoustic canal, tympanic membrane, or bones of the middle ear may be dysfunctional. Conductive hearing loss may be congenital or caused by trauma, severe otitis media, otosclerosis, neoplasms, or atresia of the ear canal. Some conductive hearing loss can be treated surgically with tympanoplasty or stapedectomy, and the use of hearing aids and assistive listening devices may also be beneficial.
Sensorineural hearing loss occurs when the sensory receptors of the inner ear are dysfunctional. Sensorineural deafness is a lack of sound perception caused by a defect in the cochlea and/or the auditory division of the vestibulocochlear nerve. This type of hearing loss is more common than conductive hearing loss and is typically irreversible. It tends to be unevenly distributed, with greater loss at higher frequencies. Sensorineural hearing loss may result from congenital malformation of the inner ear, intense noise, trauma, viral infections, ototoxic drugs (e.g., cisplatin, salicylates, loop diuretics), fractures of the temporal bone, meningitis, ménière's disease, cochlear otosclerosis, aging (i.e., presbycusis), or genetic predisposition, either alone or in combination with environmental factors. Many patients with sensorineural hearing loss can be habilitated or rehabilitated with the use of hearing aids. Patients with profound bilateral sensorineural hearing loss (e.g., at least 90 dB) who derive no benefit from conventional hearing aids may be appropriate candidates for the cochlear implant device, which bypasses the damaged structures of the cochlea and stimulates the function of the auditory nerve. Auditory brainstem implants, which are similar to multichannel cochlear implants, are used in patients with neurofibromatosis type 2 following vestibular schwannoma removal, especially those individuals who have lost integrity of the auditory nerves.
Auditory Neuropathy (AN) is a type of sensorineural hearing loss that can be congenital or acquired. Unlike other types of sensorineural hearing loss where both Otoacoustic Emissions (OAE) and Auditory Brainstem Response (ABR) tests are likely to be abnormal, Auditory Neuropathy is characterized by normal OAE results and significantly abnormal ABR responses, even when measured with very loud sounds. The combination of normal OAE responses and severely impaired ABR responses is thought to reflect normal outer hair cell (OHC) function in the cochlea and abnormal auditory nerve function. The site of lesion for AN is often unknown, but possibilities include cochlear inner hair cells, cochlear spiral ganglia, synapse and/or eighth nerve fiber disorders. Audiograms of children with AN vary from hearing in the normal range with complaints of difficulty hearing in background noise to profound hearing loss.
Individuals with mixed hearing loss have both conductive and sensory dysfunction. Mixed hearing loss is due to disorders that can affect the middle and inner ear simultaneously, such as otosclerosis involving the ossicles and the cochlea, head trauma, middle ear tumors, and some inner ear malformations. Trauma resulting in temporal bone fractures may be associated with conductive, sensorineural, and mixed hearing loss.
Auditory Processing Disorder (APD) is a deficit in neural processing of auditory stimuli that is not due to higher order language, cognitive, or related factors. However, APD may lead to or be associated with difficulties in higher order language, learning, and communication functions. This type of auditory problem affects more complex auditory processes, such as understanding speech when there is background noise. The results of hearing sensitivity and physiological tests, such as otoacoustic emissions (OAE) and auditory brainstem response (ABR) are normal in children with a central auditory disorder.
A great many, including what exactly constitutes an APD, with some professionals being still unconvinced that it exists as a separate clinical entity, poor understanding of the boundaries and overlap between APD and language or other developmental disorders, and lack of uniform accepted guidelines regarding testing and management of APD.
Configuration of hearing loss
There are four general configurations of hearing loss:
1. Flat: thresholds essentially equal across test frequencies.
2. Sloping: lower (better) thresholds in low-frequency regions and higher (poorer) thresholds in high-frequency regions.
3. Rising: higher (poorer) thresholds in low-frequency regions and lower (better) thresholds in higher-frequency regions.
4. Trough-shaped ("cookie-bite" or "U" shaped): greatest hearing loss in the mid-frequency range, with lower (better) thresholds in low- and high-frequency regions.