Neuroimaging is employed to verify the presence of cerebral atrophy. In cases of suspected CGS; testing for XLA is possible. Differential diagnosis
This is long and includes

Arts syndrome
Autosomal recessive nonsyndromic sensorineural deafness type DFNB
Friedreich ataxia
MELAS syndrome
Mitochondrial DNA depletion syndrome (encephalomyopathic form with methylmalonic aciduria)
McLeod neuroacanthocytosis syndrome
Pendred syndrome
Usher syndrome type 1 and 2
Wolfram syndrome
X-linked spinocerebellar ataxia type 3 and 4

Treatment
Symptomatic Treatment
The treatment for Mohr-Tranebjærg vary depending on the symptoms and the evolution of these symptoms over time. Since hearing loss is prevalent in those with Mohr-Tranebjærg, hearing aids, devices, or cochlear implants are considered for increasingly serious cases. Immunoglobin
In those patients with secondary complications from the syndrome, intravenous immunoglobin may act to prevent infections in X-linked agammaglobulinemia, an inherited immune system disorder that can reduce the ability to fight infections. Similarly, those with XLA should avoid live viral vaccines. Patients that reach adulthood should seek regular evaluations from a neurologist to test the advancement of possible dementia and psychiatric manifestations

Genetic Counseling
Since the pattern of inheritance for Mohr-Tranebjærg is known to be X-linked recessive, genetic counseling is considered beneficial to families with known carriers. In the case that a female is a carrier, the statistical risk of male offspring inheriting the disease is 50%, while the probability that a female offspring will be a carrier is 50%. However, when a male is the carrier, the probability of having a female carrier offspring is 100%, and male offspring will not have any chance of inheriting the syndrome.
Because of the statistical probabilities that each sex inherits the syndrome, males are affected by Mohr-Tranebjærg more frequently than females are. Females who carry one copy of the TIMM8A gene (described in Genetics) are usually unaffected, however, may develop mild hearing loss and dystonia. Prognosis
Prognosis is poor. The combination of deafness and blindness severely affects communication, while the ongoing movement disorder results in an increasingly unstable gait. Life expectancy is highly variable and can range from death in the teenage years (after a rapidly progressive dystonia) to those that live into their 60s. History
This condition was first described in 1960. Epidemiology
Mohr-Tranebjᴂrg syndrome (MTS) prevalence is unknown. More than 90 cases (in 37 families) are known, but not all cases have been reported in the literature. See also
Mitochondrial disorders
TIMM13 and TIMM8A

References
External links

GeneReviews/NCBI/NIH/UW entry on Deafness–Dystonia–Optic Neuronopathy Syndrome
MTS — a page at NIH website