1naresh2naresh<pre>Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => Controlled
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

    [47c00bcf-bd02-4b84-ae6f-da883db64dd5] => Array
        (
            [runtime-id] => 47c00bcf-bd02-4b84-ae6f-da883db64dd5
            [type] => toll-free-key
            [service-id] => ajnr-ac.highwire.org
            [access-type] => Controlled
            [privilege] => Array
                (
                    [2cecff31-7178-4ed1-b4b0-c042705c4f81] => Array
                        (
                            [runtime-id] => 2cecff31-7178-4ed1-b4b0-c042705c4f81
                            [type] => toll-free-key
                        )

                )

            [credentials] => Array
                (
                    [method] => toll-free-key
                    [value] => tf_ipsecsha;ee1c09a0430265d4e01a6ed4855b311c6074242f
                )

        )

)
1naresh2naresh<pre>Array
(
    [urn:ac.highwire.org:guest:identity] => Array
        (
            [runtime-id] => urn:ac.highwire.org:guest:identity
            [type] => guest
            [service-id] => ajnr-ac.highwire.org
            [access-type] => FreeToRead
            [privilege] => Array
                (
                    [urn:ac.highwire.org:guest:privilege] => Array
                        (
                            [runtime-id] => urn:ac.highwire.org:guest:privilege
                            [type] => privilege-set
                            [privilege-set] => GUEST
                        )

                )

            [credentials] => Array
                (
                    [method] => guest
                )

        )

    [b9ac29f5-3bb2-4969-b178-20a26891f7fe] => Array
        (
            [runtime-id] => b9ac29f5-3bb2-4969-b178-20a26891f7fe
            [type] => toll-free-key
            [service-id] => ajnr-ac.highwire.org
            [access-type] => FreeToRead
            [privilege] => Array
                (
                    [93f2cc69-dc8a-4383-8ef7-b9bb164f9e60] => Array
                        (
                            [runtime-id] => 93f2cc69-dc8a-4383-8ef7-b9bb164f9e60
                            [type] => toll-free-key
                        )

                )

            [credentials] => Array
                (
                    [method] => toll-free-key
                    [value] => tf_ipsecsha;ee1c09a0430265d4e01a6ed4855b311c6074242f
                )

        )

)
PT  - JOURNAL ARTICLE
AU  - Elmaleh-Bergès, M.
AU  - Baumann, C.
AU  - Noël-Pétroff, N.
AU  - Sekkal, A.
AU  - Couloigner, V.
AU  - Devriendt, K.
AU  - Wilson, M.
AU  - Marlin, S.
AU  - Sebag, G.
AU  - Pingault, V.
TI  - Spectrum of Temporal Bone Abnormalities in Patients with Waardenburg Syndrome and &lt;em&gt;SOX10&lt;/em&gt; Mutations
AID  - 10.3174/ajnr.A3367
DP  - 2013 Jun 01
TA  - American Journal of Neuroradiology
PG  - 1257--1263
VI  - 34
IP  - 6
4099  - http://www.ajnr.org/content/34/6/1257.short
4100  - http://www.ajnr.org/content/34/6/1257.full
SO  - Am. J. Neuroradiol.2013 Jun 01; 34
AB  - BACKGROUND AND PURPOSE: Waardenburg syndrome, characterized by deafness and pigmentation abnormalities, is clinically and genetically heterogeneous, consisting of 4 distinct subtypes and involving several genes. SOX10 mutations have been found both in types 2 and 4 Waardenburg syndrome and neurologic variants. The purpose of this study was to evaluate both the full spectrum and relative frequencies of inner ear malformations in these patients. MATERIALS AND METHODS: Fifteen patients with Waardenburg syndrome and different SOX10 mutations were studied retrospectively. Imaging was performed between February 2000 and March 2010 for cochlear implant work-up, diagnosis of hearing loss, and/or evaluation of neurologic impairment. Eleven patients had both CT and MR imaging examinations, 3 had MR imaging only, and 1 had CT only. RESULTS: Temporal bone abnormalities were bilateral. The most frequent pattern associated agenesis or hypoplasia of ≥1 semicircular canal, an enlarged vestibule, and a cochlea with a reduced size and occasionally an abnormal shape, but with normal partition in the 13/15 cases that could be analyzed. Three patients lacked a cochlear nerve, bilaterally in 2 patients. In addition, associated abnormalities were found when adequate MR imaging sequences were available: agenesis of the olfactory bulbs (7/8), hypoplastic or absent lacrimal glands (11/14), hypoplastic parotid glands (12/14), and white matter signal anomalies (7/13). CONCLUSIONS: In the appropriate clinical context, bilateral agenesis or hypoplasia of the semicircular canals or both, associated with an enlarged vestibule and a cochlear deformity, strongly suggests a diagnosis of Waardenburg syndrome linked to a SOX10 mutation. CNCcochlear nerve canalPCWHperipheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, Hirschsprung diseaseSCCsemicircular canalsSNHLsensorineural hearing lossWSWaardenburg syndromeWS1 to WS4Waardenburg syndrome types 1–4