People with CdLS may have very tiny structures and testing may be difficult. It is advisable to consult an audiologist and/or otolaryngologist who is familiar with CdLS or who is experienced in working with infants. Pharyngeal-esophageal tubes may be useful for middle-ear drainage as needed but a physician experienced in working with small infants is usually necessary.

If a hearing loss is suspected, headsets and hearing aids should be prescribed for infants and children. Even a mild hearing loss can result in a speech and language delay. Smaller aids are available so it is not necessary or advisable to use an adult-sized aid. If the child will not leave on the aid, an audiologist or behavioural therapist may be helpful. Appropriate audiological management should include selection and fitting of suitable amplification for all listening environments. While the child's personal hearing aid may be sufficient some of the time, the use of FM amplification may be necessary in other situations.

Almost all children with CdLS are diagnosed with mild to moderate and sometimes severe hearing loss, however interviews with caretakers reveal unexpected reports regarding the history of audiological results. Many caretakers report that their children were diagnosed as severely hearing impaired at birth, moderately impaired at 12 months, and mildly impaired or without impairment at age 2 years. Since it is unusual for hearing to improve rather than worsen, it seems correct audiological assessment is difficult. Individuals with CdLS may have narrow ear canals and difficult behaviours, making examinations a challenge.

Parents often report their children seem to hear much better than their test results would indicate. Considerable confusion exists regarding hearing ability for some children. Many children fitted with hearing aids will rot tolerate the use of them or do so only sporadically. For these individuals, retesting is often important to insure that the aid is beneficial. There have also been reports of young children fitted with adult-sized hearing aids when child-sized aids are available and more appropriate.