MANAGEMENT OF ANSD

The management strategies utilized in individuals with AD are those that are originally meant for
conditions other than frequency discrimination since frequency discrimination is not affected in AN type
hearing loss . Management techniques specifically designed for this population are sparse.
Brown (2002) guidelines for management of auditory dys-synchrony suggest that individuals,
especially children, benefit from an individualized treatment plan. The major treatment options for
individuals with ANSD includes:-
- Amplification
- Cochlear implants
- FM systems
- Cued speech
- Auditory training
- Communication strategies
Amplification
Zeng et al. (1999) proposed that a special type of speech processing hearing aid is needed for people
with auditory neuropathy. In addition to amplifying sound the hearing aid should compensate for the
impaired temporal processing at suprathreshold levels.
Starr (1996) found that none of the subjects with auditory neuropathy reported to benefit from
conventional amplification. Hearing aids with a high frequency emphasis might serve to emphasize the high
frequency transient speech sounds, particularly, the high- frequency consonant.
Rance et al. (1999) showed that approximately 50% of affected children benefit from amplification.
Hood (1998) recommended high quality, low gain, wide dynamic range compression hearing aids. This
approach is intended to minimize any deleterious effects of amplification on OAEs.
Zeng and Liu (2006) suggested that following schemes could improve speech discrimination results:
- Expand temporal modulation because one of the features in clear speech is enhanced
amplitude modulation
- Filter out low frequency signals and/or shift them to high-frequency regions since patients
with AN have relatively normal pitch processing at high frequencies.
Cochlear Implant
The efficacy of cochlear implantation could be questioned for these patients with ANSD because of
the suggested pathology of the AN; however, research has supported the use of electrical stimulation in
cases of auditory nerve dysfunction.
Brown (2002) has suggested that the candidacy cannot be determined on the basis of pure-tone
hearing levels. Rather, the auditory discrimination abilities of the child is used for candidacy determination.
Those who succeed with a cochlear implant are the children for whom the site of lesion resides in the
cochlea or the transmission of information from the cochlea to the auditory nerve. If the probable cause of
the disorder is a true neuropathy of the auditory nerve the child would not be a good candidate for a cochlear
implant.
Abbas (1995) reported that electrical stimulation produces synchronous ABRs in the presence of
peripheral auditory nerve demyelination. This suggested that the electrical stimulation of a cochlear implant
could provide reliable, consistent neural firing even in the presence of a diseased peripheral nerve. If
electrical stimulation can normalize the timing of firing patterns in the auditory nerve, the results should be
better speech perception. In addition, electrical stimulation provided by the cochlear implant may even
promote neural survival (Araki et al., 1998) and restore temporal encoding (Shannon, 1993)
FM systems
Patients with auditory neuropathy experience poorer speech perception in background noise. FM
systems have been proved to be an effective treatment for auditory processing disorders (Stach et al, 1987)
as they improve the signal to noise ratio.
Considering the increased risk of difficulty hearing in the presence of background noise, Roush
(2008) suggested that the personal FM use by parents and other caregivers is likely to be beneficial and FM
use in the classroom is especially important.
There are several reasons why FM systems may be helpful in some AN /AD patients.
- Removing interference from noise would be beneficial in allowing patients to maximize
ability to use their residual speech recognition ability.
- Efferent feedback function is disabled in AN/AD patients due to compromise of afferent
function (Hood et al., 2003). According to anti-masking model proposed by Liberman (1998),
the efferent feedback function is thought to assist in listening to noise.
- Some AN/AD patients with residual speech recognition ability in quiet report that FM
systems are helpful when listening in background noise (Hood, Wilensky, Li, Berlin, 2003).
Cued Speech
Visual modes of communication would appear to be a logical choice to supplement auditory
presentation of spoken language in some cases.
One method of visual support of spoken language is cued speech. Cued speech is a method for
providing a manual- visual aid to lip reading. Cued speech was developed by Cornett (1967) as a manual
representation of phonological cues that are used simultaneously with oral speech.
The phonological representations are depicted by a series of hand-shapes that are made in various
positions at the speaker’s mouth or at the level of the larynx. It has been suggested that cued speech can aid
in the development of phonological representation of language for children who are hearing impaired
(Charlier, 1992). Cued speech has been used in conjunction with oral-aural methods of language
development, with total communication and with signed language.
Auditory training
An auditory training program needs to be used if and when the child starts to demonstrate the ability
to listen to and process auditory stimuli. When a child can use audition to learn language, the auditory
training program is useful to identify discrete goals for therapy and to monitor success. A traditional,
structured auditory training program should also be used when a child receives a cochlear implant.
Berlin et al. (2002) has suggested that the AT is very successful with children who have synchronous
cochlear responses with hearing aids and AN/AD children who receive implants.
Vijay kumar (2010) reported that in individuals with AD improvements were seen using fine-
grained speech identification training. The improvements were seen for bisyllabic word identification scores,
identification of voiced- voiceless stops.
Speech reading
It is an important component of audiologic rehabilitation. It involves watching the speaker’s mouth,
facial expressions and hand gestures as much as possible and having awareness of the topic of conversation
and contextual cues.
Communication strategies
There are two main types: a) Anticipatory strategies b) Repair strategies
a) Anticipatory strategies involve predicting possible problems in a situation and planning ways to deal
with them. It includes
i) Selecting appropriate seating
ii) Adequate lighting
iii) Avoiding noisy situations
iv) Removing visual distractors
v) Requesting the speaker to speak naturally
b) Repair strategies are used after a breakdown in communication takes place. They are generally used
for clarification of the message. They includes
i) Asking the speaker to repeat all or part of message
ii) Asking the speaker to rephrase or simplify the message
iii) Asking a follow up question to either confirm the content of a previous message
iv) Summarizing
v) Seeking clarification
vi) Writing the message
Roush (2008): Because of the complexity of the disorder and the likelihood of additional disabilities
in children with ANSD, it is essential that evaluation and management occur in a setting where a specialized
team approach includes pediatric audiologists, speech-language pathologists, and early-intervention
specialists experienced in the management of ANSD and physicians familiar with both typical sensorineural
hearing loss and the unique needs of children with ANSD.
It is important to make sure families understand the heterogeneous nature of the disorder and its
underlying etiologies, the controversies that exist regarding treatment options, and the difficulty predicting
outcomes during infancy.